GLUTARIC ACID (GLUTARK AST)

CAS No. : 110-94-1

EC No. : 203-817-2

Synonyms:

GLUTARIC ACID; Pentanedioic acid; 110-94-1; 1,5-Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentandioic acid; glutarate; n-Pyrotartaric acid; UNII-H849F7N00B; propane-1,3; dicarboxylic acid;Glutaric acid, 99%; HSDB 5542; NSC 9238; EINECS 203-817-2; BRN 1209725; AI3-24247; CHEBI:17859; NSC9238; JFCQEDHGNNZCLN-UHFFFAOYSA-N; H849F7N00B; MFCD00004410; DSSTox_CID_1654; DSSTox_RID_76266; DSSTox_GSID_21654; CAS-110-94-1; Glutarsaeure; Pentandioate; Carboxylic acids, C6-18 and C5-15-di-; Carboxylic acids, di-, C4-6; 1czc; 1,5-Pentanedioate; PubChem18989; 4lh3;1,3-Propanedicarboxylate; WLN: QV3VQ; (C4-C6) Dibasic acids; bmse000406; SCHEMBL7414; 4-02-00-01934 (Beilstein Handbook Reference; KSC178G2T; Pentanedioic acid Glutaric acid; glutaric acid; glutarik asit; gluterik asit; glutarik asid; gluteric asit; glutaric asit; glutaric acit;GLUTARIC ACID; GLUTARIK ASIT; GLUTARK AST; GLUTARK ASD; GLUTERC AST; GLUTARC ACT; glutaric acid, calcium salt; glutaric acid, copper(2+) salt (1:1); glutaric acid, disodium salt; glutaric acid, ion(1-); glutaric acid, monosodium salt; Pentanedioic acid; 110-94-1; 1,5-Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentandioic acid; glutarate; n-Pyrotartaric acid; UNII-H849F7N00B; propane-1,3-dicarboxylic acid; Carboxylic acids, di-, C4-6; Glutaric acid, 99%; HSDB 5542; Carboxylic acids, C6-18 and C5-15-di-; NSC 9238; EINECS 203-817-2; MFCD00004410; (C4-C6) Dibasic acids; BRN 1209725; AI3-24247; CHEBI:17859; Glutarsaeure; Pentandioate; 1czc; 1,5-Pentanedioate; EINECS 273-081-5; PubChem18989; 4lh3; 1,3-Propanedicarboxylate; 1,3-Propanedicarboxylic acid ChEBI; 1,5-Pentanedioic acid ChEBI; Glutarsaeure ChEBI; Pentanedioic acid ChEBI; 1,3-Propanedicarboxylate Generator; 1,5-Pentanedioate Generator; Pentanedioate Generator; Glutarate Generator; Pentandioate; Glutaric acid Pentanedioic acid 110-94-1 1,5-Pentanedioic acid 1,3-Propanedicarboxylic acid; glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; GLUTARIC ACIDE; GLUTARDE ACT; GLUTART AST; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; glutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARK AST; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARC ACT; GLUTARAL AST; GLUTARK AST; DCARBOXYL AST; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAK AST; PENTANEDOK AST; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARK ASDÜTE; glutarik asidide;glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; GLUTARIC ACIDE; GLUTARDE ACT; GLUTART AST; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; glutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARK AST; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARC ACT; GLUTARAL AST; GLUTARK AST; DCARBOXYL AST; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAK AST; PENTANEDOK AST; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARK ASDÜTE; glutarik asidide;glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; GLUTARIC ACIDE; GLUTARDE ACT; GLUTART AST; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; glutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARK AST; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARC ACT; GLUTARAL AST; GLUTARK AST; DCARBOXYL AST; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAK AST; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARK ASDÜTE; glutarik asidide; glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; GLUTARIC ACIDE; GLUTARDE ACT; GLUTART AST; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; glutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAK AST; PENTANEDOK AST; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARK ASDÜTE; glutarik asidide; glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; GLUTARIC ACIDE; GLUTARDE ACT; GLUTART AST; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; glutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARK AST; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARC ACT; GLUTARAL AST; GLUTARK AST; DCARBOXYL AST; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARK ASDÜTE; glutarik asidide; glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAK AST; PENTANEDOK AST; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid; Pentandioic acid; GLUTARK ASDÜTE; glutarik asidide; glutaric acid; glatarc asit; gulutaric asit; grutarc acide; glutarc acde; GLUTARIC ACIDE; GLUTARDE ACT; GLUTART AST; gulutarik asit; glikol asit; glutarol acit; glutran acit; glutaric acit; klutaric asit; glutaric acid; glutarik asit; glutarik asit; gulutaric acid; GLUTARIC ACID; KLUTARK AST; glutarik asit; acit glutaral; glutaricasit; glutaraldeyde acit; gulutaik acit; asit glutaral; glutaric acid; GLUTARC ACT; GLUTARAL AST; DCARBOXYL AST; dicarboxyl asit; Pentanedioic Acid; 1,3; Pentanedioic Acid; 1,3-Propanedicarboxylic Acid; 1,5-Pentanedioic Acid; Glutaric Anhydride; klutarik asit anhidrit; glutaric acide anhydrous; GLUTARK AST ANHDRT; Glutaric Acid-2-methylamino-5-nitromonoanilide; Glutaric Acid-1,5-13C2; Pentanedioic acid; GLUTAK AST; PENTANEDOK AST; phentonaik asit; PENTADIOIC ACID; 1,3-Propandicarbox ylate; 1,5-pentandioat; Asit glutarique; Glutarsäure; Asit glutarique; Glutarsäure; Pentanedioic acid; Pentanedioic acid; 1,3-Propanedicarboxylic acid; Pentanedioic acid; n-Pyrotartaric acid

Glutaric Acid

Glutaric acid (glutarik asit) is a simple five-carbon linear dicarboxylic acid. Glutaric acid (glutarik asit) is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid (glutarik asit) may cause irritation to the skin and eyes. When present in sufficiently high levels, Glutaric acid (glutarik asit) can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of Glutaric acid (glutarik asit) are associated with at least three inborn errors of metabolism, including Glutaric acid (glutarik asit)uria type I, malonyl-CoA decarboxylase deficiency, and Glutaric acid (glutarik asit)uria type III. Glutaric acid (glutarik asit)uria type I (Glutaric acid (glutarik asit)emia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1. 3. 99. 7, GCDH). Excessive levels of their intermediate breakdown products (e. g. Glutaric acid (glutarik asit), glutaryl-CoA, 3-hydroxyGlutaric acid (glutarik asit), glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with Glutaric acid (glutarik asit)emia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because Glutaric acid (glutarik asit), like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7. 35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated Glutaric acid (glutarik asit)uria. Many affected children with organic acidemias experience intellectual disability or delayed development. In adults, acidosis or acidemia is characterized by headaches, confusion, feeling tired, tremors, sleepiness, and seizures. Treatment of Glutaric acid (glutarik asit)uria is mainly based on the restriction of lysine intake, supplementation of carnitine, and an intensification of therapy during intercurrent illnesses. The major principle of dietary treatment is to reduce the production of Glutaric acid (glutarik asit) and 3-hydroxyGlutaric acid (glutarik asit) by restriction of natural protein, in general, and of lysine, in particular (PMID: 17465389, 15505398).

Production of Glutaric acid (glutarik asit)

Glutaric acid (glutarik asit) can be prepared by the ring-opening of butyrolactone with potassium cyanide to give the mixed potassium carboxylate-nitrile that is hydrolyzed to the diacid.[1] Alternatively hydrolysis, followed by oxidation of dihydropyran gives Glutaric acid (glutarik asit). It can also be prepared from reacting 1,3-dibromopropane with sodium or potassium cyanide to obtain the dinitrile, followed by hydrolysis.

Uses of Glutaric acid (glutarik asit)

1,5-Pentanediol, a common plasticizer and precursor to polyesters is manufactured by hydrogenation of Glutaric acid (glutarik asit) and its derivatives.[2]

Glutaric acid (glutarik asit) itself has been used in the production of polymers such as polyester polyols, polyamides. The odd number of carbon atoms (i.e. 5) is useful in decreasing polymer elasticity.[citation needed]

Uvitonic acid is obtained by the action of ammonia on Glutaric acid (glutarik asit).

Safety

Glutaric acid (glutarik asit) may cause irritation to the skin and eyes.[3] Acute hazards include the fact that this compound may be harmful by ingestion, inhalation or skin absorption.

Application of Glutaric acid (glutarik asit)

Glutaric acid (glutarik asit) may be employed as starting reagent in the synthesis of glutaric anhydride.

Glutaric acid (glutarik asit) may be used for the following studies:

• Complexation with DL-lysine. Complexes have been reported to possess zwitterionic lysinium ions (positively charged) and semi-glutarate ions (negatively charged).[8]

• Synthesis of complexes with L-arginine and L-histidine.[7]

• Preparation of glycine-Glutaric acid (glutarik asit) co-crystals. Phase transition studies of these cocrystals have been reported by single-crystal X-ray diffraction, polarized Raman spectroscopy and differential scanning calorimetry.[1]

General description

Glutaric acid (glutarik asit) (Pentanedioic Acid) is a linear dicarboxylic acid. It has been prepared by oxidizing cyclopentane, cyclopentanol and cyclopentanone.[9]

Glutaric acid (glutarik asit) is a pentanedioic acid. On exposure to X-rays, Glutaric acid (glutarik asit) crystals generate two stable free radicals. These free radicals have been investigated by electron nuclear double resonance (ENDOR) technique.[5] Presence of Glutaric acid (glutarik asit) in urine and plasma is an indicator of type I Glutaric acid (glutarik asit)uria (GA-I).[6]

Glutaric acid (glutarik asit) is formed as an intermediate during the catabolism of lysine in mammals.[3] Electron spin resonance spectra of radical (CO2H)CH2CH2CH(CO2H formed in Glutaric acid (glutarik asit) crystal after γ-irradiation is reported to remains trapped in it.[2] Polymorphism of Glycine-Glutaric acid (glutarik asit) co-crystals has been studied by single crystal X-ray diffraction and Raman spectroscopy.[4]

Low-temperature phase transition in glycine-Glutaric acid (glutarik asit) co-crystals studied by single-crystal X-ray diffraction, Raman spectroscopy and differential scanning calorimetry.

Glutaric acid (glutarik asit)

Glutaric acid (glutarik asit)uria type 1 (OMIM #231670) due to glutaryl-coenzyme A dehydrogenase deficiency is associated with accumulation of Glutaric acid (glutarik asit), glutaryl carnitine, and secondary metabolites in body fluids. The clinical picture is variable. Most patients are macrocephalic.

The authors selected the Glutaric acid (glutarik asit) cocrystal2 for further evaluation because of the relatively high melting point of the cocrystal and the expected high water solubility of the cocrystal because of the high water solubility of the coformer. A solvent-based

Glutaric acid (glutarik asit)uria Type 1 (OMIM 231670)

The metabolism of lysine, hydroxylsine, and tryptophan is disrupted secondary to deficiency in glutaryl-CoA dehydrogenase, a mitochondrial enzyme. This results in the accumulation of Glutaric acid (glutarik asit) and 3-hydroxyGlutaric acid (glutarik asit).

Investigation

Neuroimaging is characteristic, with frontotemporal atrophy and often subdural effusions or hematomas. This may lead to initial suspicion of child abuse. There is excessive glutaric and 3-hydroxyGlutaric acid (glutarik asit) in the urine. Plasma free carnitine is reduced and glutaryl carnitine is elevated. Reduced enzyme activity is demonstrated in fibroblasts.

Urine organic acid analysis detects a wide range of compounds. It is an excellent diagnostic test for the organic acidemias involving propionic, methylmalonic, and isovaleric acids. It also detects Glutaric acid (glutarik asit), which is a progressive neurotoxic defect in biomolecule conversion. The fatty acid oxidation defects also result in abnormal compounds in the urine. The presence of succinylacetone is a hallmark of tyrosinemia; similarly, the presence of isoleucine metabolites is a hallmark of maple syrup urine disease. Lactic acid and ketones are also detectable on organic acid analysis but are not always well correlated with plasma levels.

Common Name Glutaric acid (glutarik asit)

Class Small Molecule

Description Glutaric acid (glutarik asit) is a simple five-carbon linear dicarboxylic acid. Glutaric acid (glutarik asit) is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Glutaric acid (glutarik asit) may cause irritation to the skin and eyes. When present in sufficiently high levels, Glutaric acid (glutarik asit) can act as an acidogen and a metabotoxin. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of Glutaric acid (glutarik asit) are associated with at least three inborn errors of metabolism, including Glutaric acid (glutarik asit)uria type I, malonyl-CoA decarboxylase deficiency, and Glutaric acid (glutarik asit)uria type III. Glutaric acid (glutarik asit)uria type I (Glutaric acid (glutarik asit)emia type I, glutaryl-CoA dehydrogenase deficiency, GA1, or GAT1) is an inherited disorder in which the body is unable to completely break down the amino acids lysine, hydroxylysine, and tryptophan due to a deficiency of mitochondrial glutaryl-CoA dehydrogenase (EC 1.3.99.7, GCDH). Excessive levels of their intermediate breakdown products (e.g. Glutaric acid (glutarik asit), glutaryl-CoA, 3-hydroxyGlutaric acid (glutarik asit), glutaconic acid) can accumulate and cause damage to the brain (and also other organs). Babies with Glutaric acid (glutarik asit)emia type I are often born with unusually large heads (macrocephaly). Macrocephaly is amongst the earliest signs of GA1. GA1 also causes secondary carnitine deficiency because Glutaric acid (glutarik asit), like other organic acids, is detoxified by carnitine. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to h …Read more

Mechanism of Toxicity Accumulation of Glutaric acid (glutarik asit) in the body has been shown to be toxic. The accumulation of Glutaric acid (glutarik asit) ranging from slightly or intermittently elevated urinary Glutaric acid (glutarik asit) to gross organic aciduria occurs in Glutaric acid (glutarik asit)uria. Glutaric acid (glutarik asit)uria type 1 is an autosomal-recessive disorder resulting from a deficiency of mitochondrial glutaryl-CoA dehydrogenase which is involved in the metabolism of lysine, hydroxylysine,

Uses/Sources This is an endogenously produced metabolite found in the human body. It is used in metabolic reactions, catabolic reactions or waste generation.

Minimum Risk Level Not Available

Health Effects Chronically high levels of Glutaric acid (glutarik asit) are associated with at least 3 inborn errors of metabolism including: Glutaric acid (glutarik asit)uria Type I and Glutaric acid (glutarik asit)uria Type III.

Clinical Information

Acylcarnitine analysis is included in newborn screening blood testing and is utilized for detection of several inborn errors of metabolism, including fatty acid oxidation disorders (FAOD) and organic acidemias (OA). A limitation of this analytic method is its inability to differentiate between several isomers. Additional testing of 2-hydroxy Glutaric acid (glutarik asit) (2OH-GA), 3-hydroxy Glutaric acid (glutarik asit) (3OH-GA), Glutaric acid (glutarik asit) (GA), methylsuccinic acid (MSA), and ethylmalonic acid (EMA) by LC-MS/MS allows better differentiation among C4-acylcarnitine and glutarylcarnitine/C10-OH isomers.

Glutarylcarnitine (C5-DC) is elevated in Glutaric acid (glutarik asit)emia type 1 (GA-1), but is not differentiated from C10-OH acylcarnitine. GA-1, is caused by a deficiency of glutaryl-CoA dehydrogenase and is characterized by bilateral striatal brain injury leading to dystonia, often a result of acute neurologic crises triggered by illness. Individuals with GA-1 typically show elevations of Glutaric acid (glutarik asit) and 3OH-GA, even in those considered to be “low excretors.”

Glutaric acid (glutarik asit)

Class Small Molecule

DRUG INTERACTION

Acetazolamide The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Acetazolamide.

Acetylsalicylic acid The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Acetylsalicylic acid.

Acyclovir The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Acyclovir.

Adefovir dipivoxil The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Adefovir dipivoxil.

Allopurinol The excretion of Allopurinol can be decreased when combined with Glutaric acid (glutarik asit).

Alprostadil The excretion of Alprostadil can be decreased when combined with Glutaric acid (glutarik asit).

Aminohippuric acid The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Aminohippuric acid.

Aminophenazone The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Aminophenazone.

Amoxicillin The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Amoxicillin.

Antipyrine The excretion of Glutaric acid (glutarik asit) can be decreased when combined with Antipyrine.

A limitation of this analytic method is its inability to differentiate between several isomers. Additional testing of 2-hydroxy Glutaric acid (glutarik asit) (2OH-GA), 3-hydroxy Glutaric acid (glutarik asit) (3OH-GA), Glutaric acid (glutarik asit) (GA), methylsuccinic acid (MSA), and ethylmalonic acid (EMA) by LC-MS/MS allows better differentiation among C4-acylcarnitine and glutarylcarnitine/C10-OH isomers.

Glutaric acid (glutarik asit)emia (GA-2), also known as multiple acyl-CoA dehydrogenase deficiency (MADD), is caused by defects in either the electron transfer flavoprotein (ETF) or ETF-ubiquinone oxidoreductase. This disease can be severe and is often fatal in the first weeks of life, with typical symptoms of hypoglycemia, muscle weakness, metabolic acidosis, dysmorphic features, cardiac defects or arrhythmias, renal cysts, and fatty infiltration of the liver. GA-2 can have a milder presentation, also known as ethylmalonic-adipic aciduria, with Reye-like illnesses in childhood and muscle weakness in childhood and adulthood. In addition to elevations in Glutaric acid (glutarik asit), individuals with GA-2 can also show increased EMA, MSA, and 2OH-GA.

Reference Values

2-OH Glutaric acid (glutarik asit): < or =25 nmol/mL

3-OH Glutaric acid (glutarik asit): < or =1.5 nmol/mL

Glutaric acid (glutarik asit): < or =1.5 nmol/mL

Methylsuccinic acid: < or =0.45 nmol/mL

Ethylmalonic acid: < or =3.5 nmol/mL

Normal levels of EMA in the context of elevated C4 is consistent with a diagnosis of isobutyryl-CoA dehydrogenase (IBDH) deficiency.

Elevation of Glutaric acid (glutarik asit) (GA) and 3-hydroxy Glutaric acid (glutarik asit) (3OH-GA) are consistent with a diagnosis of Glutaric acid (glutarik asit)emia type 1 (GA-1).

Elevation of GA, 2-hydroxy Glutaric acid (glutarik asit) (2OH-GA), 3OH-GA, EMA, and MSA are consistent with a diagnosis of Glutaric acid (glutarik asit)emia (GA-2).

2. Kolker S, Christensen E, Leonar JV, et al: Diagnosis and management of Glutaric acid (glutarik asit)uria type I-revised recommendations. J Inherit Metab Dis 2011;34:677-694

3. Frerman FE, Goodman SI: Chapter 103: Defects of electron transfer flavoprotein and electron transfer flavoprotein-ubiquinone oxidoreductase: Glutaric acid (glutarik asit)emia Type II. In Scriver’s Online Metabolic and Molecular Bases of Inherited Disease. Edited by CR Scriver, AL Beaudet, D Valle, et al. Accessed 8/17/17. Available at Glutaric acid (glutarik asit)uria Type I

1) Glutaric acid (glutarik asit)uria IIA (GA IIA) is the neonatal form of glutaricaciduria II. This form of Glutaric acid (glutarik asit)uria II is a very rare, X-linked hereditary disorder characterized by large amounts of glutaric and other acids in blood and urine. The disorder is caused by dysfunction of the electron-transferring flavoprotein in the mitochondria.

2) Glutarica aciduria IIB (GA IIB; ethylmalonic adipicaciduria) is the adult form of glutaricaciduria II. This milder form of the disorder is inherited in an autosomal recessive pattern. Acidity of the body tissues (metabolic acidosis), and a low blood sugar level (hypoglycemia) without an elevated level of ketones in body tissues (ketosis), occur during adulthood. Large amounts of Glutaric acid (glutarik asit) in the blood and urine are caused by a deficiency of the enzyme multiple acyl-CoA dehydrogenase. (For more information on this disorder, choose “Glutaric acid (glutarik asit)uria II” as your search term in the Rare Disease Database.)

Glutaric acid (glutarik asit)uria III is an autosomal recessive genetic condition characterized by accumulation or excretion of Glutaric acid (glutarik asit) and caused by mutations in the C7ORF10 gene. Symptoms vary and some individuals show no symptoms

Goodman SI, Frerman FE. Organic acidemias due to defects in lysine oxidation: 2-ketoadipic acidemia and Glutaric acid (glutarik asit)emia. In: Scriver CR, Beaudet AL, Sly WS, et al. Eds. The Metabolic Molecular Basis of Inherited Disease. 7th ed. McGraw-Hill Companies. New York, NY; 1995:1451-60.

3.6. Effect of Glutaric acid (glutarik asit) on Caspase 3 Transcript and Protein Levels

Quantitative RT-PCR was performed to monitor mRNA expression of the apoptotic executioner caspase 3 (Figure 9(a)). The comparative method was used to analyse relative expression levels. Caspase 3 mRNA expression at 6 hours after treatment with 1, 10, 25, and 50 mM GA was upregulated about 1.40-fold, 1.67-fold, and 1.95-fold, respectively, compared to control. Thus GA might induce apoptosis via caspase 3 activation.

4. Discussion

Glutaric acid (glutarik asit)uria type I is an autosomal recessive disorder characterized by high levels of GA, 3-hydroxyGlutaric acid (glutarik asit) (3-OHGA), glutaconic acid, and glutaryl-CoA in body fluids as well as degenerative changes in the striatal and frontotemporal cortical neurons. A deficiency of cerebral GCDH activity is attributed to the development of neurological damage in GA I patients. However, the comprehension of the degeneration mechanism in the basal ganglia still remains partial.

Glutaric acid (glutarik asit) is the organic compound with the formula C3H6(COOH)2 . Although the related “linear” dicarboxylic acids adipic and succinic acids are water-soluble only to a few percent at room temperature, the water-solubility of Glutaric acid (glutarik asit) is over 50% (w/w).

Glutaric acid (glutarik asit) is naturally produced in the body during the metabolism of some amino acids, including lysine and tryptophan. Defects in this metabolic pathway can lead to a disorder called Glutaric acid (glutarik asit)uria, where toxic byproducts build up and can cause severe encephalopathy.

1,5-Pentanediol, a common plasticizer and precursor to polyesters is manufactured by hydrogenation of Glutaric acid (glutarik asit) and its derivatives.[2]

Uvitonic acid is obtained by the action of ammonia on Glutaric acid (glutarik asit).

Glutaric acid (glutarik asit) may cause irritation to the skin and eyes.[3] Acute hazards include the fact that this compound may be harmful by ingestion, inhalation or skin absorption.[3]

Glutaric acid (glutarik asit) (Pentanedioic Acid) is a linear dicarboxylic acid. It has been prepared by oxidizing cyclopentane, cyclopentanol and cyclopentanone.[9]

Application of Glutaric acid (glutarik asit)

Glutaric acid (glutarik asit) may be employed as starting reagent in the synthesis of glutaric anhydride.[9]

Chronically high levels of Glutaric acid (glutarik asit) are associated with at least three inborn errors of metabolism, including Glutaric acid (glutarik asit)uria type I

Glutaric acid (glutarik asit) has the lowest melting point among dicarboxylic acids (98 C); it is very soluble in water and the solution in water is a medium strong acid. Short-term exposure to Glutaric acid (glutarik asit) may cause irritation to the eyes, skin and the respiratory tract.

Glutarik Asit

Glutarik asit (glutaric acid), basit bir be karbonlu dorusal dikarboksilik asittir. Glutarik asit (glutaric acid), lizin ve triptofan dahil olmak üzere baz amino asitlerin metabolizmas srasnda vücutta doal olarak üretilir. Glutarik asit (glutaric acid) ciltte ve gözlerde tahrie neden olabilir. Yeterince yüksek seviyelerde bulunduunda, Glutarik asit (glutaric acid) bir asidojen ve bir metabotoksin görevi görebilir. Bir asidojen, birçok organ sistemi üzerinde çok sayda yan etkiye sahip olan asidozu indükleyen asidik bir bileiktir. Bir metabotoksin, kronik olarak yüksek seviyelerde olumsuz salk etkilerine neden olan, endojen olarak üretilen bir metabolittir. Kronik olarak yüksek Glutarik asit (glutaric acid) seviyeleri, glutarik asidüri tip I, malonil-CoA dekarboksilaz eksiklii ve glutarik asidüri tip III dahil olmak üzere en az üç doutan metabolizma hatasyla ilikilidir. Glutarik asidüri tip I (glutarik asidemi tip I, glutaril-CoA dehidrojenaz eksiklii, GA1 veya GAT1), mitokondriyal eksiklii nedeniyle vücudun amino asitleri lizin, hidroksilisin ve triptofan tamamen parçalayamad kaltsal bir hastalktr. glutaril-CoA dehidrojenaz (EC 1. 3. 99. 7, GCDH). Ara parçalanma ürünlerinin (örnein Glutarik asit (glutaric acid), glutaril-CoA, 3-hidroksiGlutarik asit (glutaric acid), glutakonik asit) ar seviyeleri birikebilir ve beyinde (ve ayrca dier organlarda) hasara neden olabilir. Glutarik asidemi tip I olan bebekler genellikle allmadk derecede büyük kafalarla (makrosefali) doarlar. Makrosefali, GA1’in ilk belirtileri arasndadr. GA1 ayrca ikincil karnitin eksikliine neden olur çünkü dier organik asitler gibi Glutarik asit (glutaric acid) de karnitin tarafndan detoksifiye edilir. Kandaki (organik asidemi), idrardaki (organik asidüri), beyindeki ve dier dokulardaki anormal derecede yüksek organik asit seviyeleri genel metabolik asidoza yol açar. Asidoz tipik olarak arteriyel pH 7’nin altna dütüünde ortaya çkar. 35. Asidozlu bebeklerde balangç semptomlar zayf beslenme, kusma, itahszlk, zayf kas tonusu (hipotoni) ve enerji eksiklii (uyuukluk) içerir. Bunlar kalp, karacier ve böbrek anormalliklerine, nöbetlere, komaya ve muhtemelen ölüme kadar ilerleyebilir. Bunlar ayn zamanda tedavi edilmemi glutarik asidüri’nin karakteristik semptomlardr. Organik asidemileri olan birçok etkilenen çocuk, zihinsel engellilik veya gecikmi geliim yaar. Yetikinlerde asidoz veya asidemi ba ars, kafa karkl, yorgunluk hissi, titreme, uyku hali ve nöbetlerle karakterizedir. Glutarik asidüri tedavisi temel olarak lizin almnn kstlanmasna, karnitin takviyesine ve birlikte görülen hastalklar srasnda tedavinin younlatrlmasna dayanr. Diyet tedavisinin ana prensibi, genel olarak doal proteinin ve özel olarak lizinin kstlanmasyla Glutarik asit (glutaric acid) ve 3-hidroksiGlutarik asit (glutaric acid) üretimini azaltmaktr (PMID: 17465389, 15505398).

Glutarik asit (glutaric acid) üretimi

Glutarik asit (glutaric acid), diasite hidrolize olan kark potasyum karboksilat-nitrili vermek için butirolaktonun potasyum siyanür ile halka açlmasyla hazrlanabilir. [1] Alternatif olarak hidroliz, ardndan dihidropiran oksidasyonu, Glutarik asit (glutaric acid) verir. Dinitril elde etmek için 1,3-dibromopropann sodyum veya potasyum siyanür ile reaksiyona sokulmas ve ardndan hidroliz yoluyla da hazrlanabilir.

Glutarik asit (glutaric acid) Kullanm Alanlar

Poliesterler için yaygn bir plastikletirici ve öncü olan 1,5-Pentandiol, Glutarik asit (glutaric acid) ve türevlerinin hidrojenasyonu ile üretilir. [2]

Polyester polioller, poliamidler gibi polimerlerin üretiminde glutarik asidin kendisi kullanlmtr. Tek sayda karbon atomu (yani 5), polimer esnekliini azaltmada yararldr. [Kaynak belirtilmeli]

Uvitonik asit, amonyan Glutarik asit (glutaric acid) üzerindeki etkisiyle elde edilir.

Emniyet

Glutarik asit (glutaric acid) ciltte ve gözlerde tahrie neden olabilir. [3] Akut tehlikeler, bu bileiin yutulmas, solunmas veya cilt tarafndan emilmesi yoluyla zararl olabilecei gerçeini içerir.

Glutarik asit (glutaric acid) uygulamas

Glutarik asit (glutaric acid), glutarik anhidrit sentezinde balangç reaktifi olarak kullanlabilir.

Glutarik asit (glutaric acid), aadaki çalmalar için kullanlabilir:

• DL-lizin ile kompleksleme. Komplekslerin zwitteriyonik lizinyum iyonlarna (pozitif yüklü) ve yar glutarat iyonlarna (negatif yüklü) sahip olduu bildirilmitir. [8]

• L-arginin ve L-histidin ile komplekslerin sentezi. [7]

• Glisin-Glutarik asit (glutaric acid) ko-kristallerinin hazrlanmas. Bu kokristallerin faz geçi çalmalar, tek kristalli X-n krnm, polarize Raman spektroskopisi ve diferansiyel taramal kalorimetri ile bildirilmitir. [1]

Genel açklama

Glutarik asit (glutaric acid) (Pentandioik Asit) dorusal bir dikarboksilik asittir. Siklopentan, siklopentanol ve siklopentanon oksitlenerek hazrlanmtr. [9]

Glutarik asit (glutaric acid) bir pentandioik asittir. X nlarna maruz kaldnda, Glutarik asit (glutaric acid) kristalleri iki kararl serbest radikal oluturur. Bu serbest radikaller elektron nükleer çift rezonans (ENDOR) teknii ile aratrlmtr. [5] drarda ve plazmada Glutarik asit (glutaric acid) varl tip I glutarik asidüri (GA-I) göstergesidir.

Memelilerde lizin katabolizmas srasnda bir ara ürün olarak Glutarik asit (glutaric acid) oluur. [3] Radikal (CO2H) CH2CH2CH (CO2H) radikalinin elektron spin rezonans spektrumlar γ-nlamadan sonra Glutarik asit (glutaric acid) kristalinde hapsolmu olarak kald. [2] Glisin-Glutarik asit (glutaric acid) ko-kristallerinin polimorfizmi tek kristal X-n ile incelenmitir. krnm ve Raman spektroskopisi. [4]

Tek kristalli X-n krnm, Raman spektroskopisi ve diferansiyel tarama kalorimetrisi ile incelenen glisin-Glutarik asit (glutaric acid) ko-kristallerinde düük scaklk faz geçii.

Glutarik asit (glutaric acid)

Glutaril-koenzime bal glutarik asidüri tip 1 (OMIM # 231670) Bir dehidrojenaz eksiklii, vücut svlarnda Glutarik asit (glutaric acid), glutaril karnitin ve ikincil metabolitlerin birikimi ile ilikilidir. Klinik tablo deikendir. Çou hasta makrosefalidir.

Yazarlar, e kristalin nispeten yüksek erime noktas ve birlikte oluturucunun yüksek suda çözünürlüü nedeniyle birlikte kristalin beklenen yüksek suda çözünürlüü nedeniyle daha fazla deerlendirme için Glutarik asit (glutaric acid) e kristalini2 seçti. Solvent bazl

Glutarik Asidüri Tip 1 (OMIM 231670)

Lizin, hidroksilsin ve triptofann metabolizmas, mitokondriyal bir enzim olan glutaril-CoA dehidrojenazdaki eksiklie bal olarak bozulur. Bu, Glutarik asit (glutaric acid) ve 3-hidroksiGlutarik asit (glutaric acid) birikimiyle sonuçlanr.

Aratrma

Nörogörüntüleme, frontotemporal atrofi ve sklkla subdural efüzyon veya hematom ile karakteristiktir. Bu, balangçta çocuk istismar üphesine yol açabilir. drarda ar glutarik ve 3-hidroksiGlutarik asit (glutaric acid) var. Plazma içermeyen karnitin azaltlr ve glutaril karnitin yükselir. Azalan enzim aktivitesi fibroblastlarda gösterilmitir.

drar organik asit analizi, geni bir bileik yelpazesini tespit eder. Propiyonik, metilmalonik ve izovalerik asitleri içeren organik asidemiler için mükemmel bir tehis testidir. Ayn zamanda, biyomolekül dönüümünde ilerleyici bir nörotoksik kusur olan glutarik asidi de tespit eder. Ya asidi oksidasyon kusurlar ayrca idrarda anormal bileiklere neden olur. Süksinil asetonun varl tirozineminin ayrt edici özelliidir; benzer ekilde, izolösin metabolitlerinin varl, akçaaaç urubu idrar hastalnn ayrt edici özelliidir. Laktik asit ve ketonlar da organik asit analizinde saptanabilir, ancak plazma seviyeleri ile her zaman iyi bir korelasyon göstermez.

Ortak sim Glutarik asit (glutaric acid)

Snf Küçük Molekül

Tanm Glutarik asit (glutaric acid), basit bir be karbonlu dorusal dikarboksilik asittir. Glutarik asit (glutaric acid), lizin ve triptofan dahil olmak üzere baz amino asitlerin metabolizmas srasnda vücutta doal olarak üretilir. Glutarik asit (glutaric acid) ciltte ve gözlerde tahrie neden olabilir. Yeterince yüksek seviyelerde bulunduunda, Glutarik asit (glutaric acid) bir asidojen ve bir metabotoksin görevi görebilir. Bir asidojen, birçok organ sistemi üzerinde çok sayda yan etkiye sahip olan asidozu tetikleyen asidik bir bileiktir. Bir metabotoksin, kronik olarak yüksek seviyelerde olumsuz salk etkilerine neden olan, endojen olarak üretilen bir metabolittir. Kronik olarak yüksek Glutarik asit (glutaric acid) seviyeleri, glutarik asidüri tip I, malonil-CoA dekarboksilaz eksiklii ve glutarik asidüri tip III dahil olmak üzere en az üç doutan metabolizma hatasyla ilikilidir. Glutarik asidüri tip I (glutarik asidemi tip I, glutaril-CoA dehidrojenaz eksiklii, GA1 veya GAT1) mitokondriyal eksiklii nedeniyle vücudun amino asitleri lizin, hidroksilisin ve triptofan tamamen parçalayamad kaltsal bir hastalktr. glutaril-CoA dehidrojenaz (EC 1.3.99.7, GCDH). Ara parçalanma ürünlerinin ar seviyeleri (örnein, Glutarik asit (glutaric acid), glutaril-CoA, 3-hidroksiGlutarik asit (glutaric acid), glutakonik asit) birikebilir ve beyne (ve ayrca dier organlara) zarar verebilir. Glutarik asidemi tip I olan bebekler genellikle allmadk derecede büyük kafalarla (makrosefali) doarlar. Makrosefali, GA1’in en erken belirtileri arasndadr. GA1 ayrca ikincil karnitin eksikliine neden olur çünkü Glutarik asit (glutaric acid), dier organik asitler gibi, karnitin tarafndan detoksifiye edilir. Kandaki (organik asidemi), idrardaki (organik asidüri), beyindeki ve dier dokulardaki anormal derecede yüksek organik asit seviyeleri genel metabolik asidoza yol açar. Asidoz tipik olarak arteriyel pH 7,35’in altna dütüünde ortaya çkar. Asidozlu bebeklerde ilk semptomlar zayf beslenme, kusma, itahszlk, zayf kas tonusu (hipotoni) ve enerji eksiklii (uyuukluk) içerir. Bunlar h’ye kadar ilerleyebilir … Daha fazla oku

Toksisite Mekanizmas Vücutta Glutarik asit (glutaric acid) birikiminin toksik olduu gösterilmitir. Glutarik asidüri, hafif veya aralkl olarak yükselmi üriner Glutarik asit (glutaric acid)ten büyük organik asidüriye kadar deien Glutarik asit (glutaric acid) birikimi, glutarik asidüri ile oluur. Glutarik asidüri tip 1, lizin, hidroksilisin metabolizmasnda rol oynayan mitokondriyal glutaril-CoA dehidrojenaz eksikliinden kaynaklanan otozomal resesif bir bozukluktur.

Kullanmlar / Kaynaklar Bu, insan vücudunda bulunan endojen olarak üretilen bir metabolittir. Metabolik reaksiyonlarda, katabolik reaksiyonlarda veya atk oluumunda kullanlr.

Minimum Risk Seviyesi Mevcut Deil

Salk Etkileri Kronik olarak yüksek Glutarik asit (glutaric acid) seviyeleri, aadakileri içeren en az 3 doutan metabolizma hatasyla ilikilidir: Glutarik Asidüri Tip I ve Glutarik Asidüri Tip III.

Klinik bilgiler

Açilkarnitin analizi, yenidoan tarama kan testine dahil edilir ve ya asidi oksidasyon bozukluklar (FAOD) ve organik asidemiler (OA) dahil olmak üzere birçok doutan metabolizma hatalarnn saptanmas için kullanlr. Bu analitik yöntemin bir snrlamas, birkaç izomer arasnda ayrm yapamamasdr. LC-MS / MS ile 2-hidroksi Glutarik asit (glutaric acid) (2OH-GA), 3-hidroksi Glutarik asit (glutaric acid) (3OH-GA), Glutarik asit (glutaric acid) (GA), metilsüksinik asit (MSA) ve etilmalonik asidin (EMA) ek testi, C4-asilkarnitin ve glutarilkarnitin / C10-OH izomerleri arasnda daha iyi farkllama.

Glutarilkarnitin (C5-DC), glutarik asidemi tip 1’de (GA-1) yükselir, ancak C10-OH açilkarnitinden farkl deildir. GA-1, glutaril-CoA dehidrojenaz eksikliinden kaynaklanr ve genellikle hastalk tarafndan tetiklenen akut nörolojik krizlerin bir sonucu olan distoniye yol açan bilateral striatal beyin hasar ile karakterizedir. GA-1’li kiiler tipik olarak, “düük boaltclar” olarak kabul edilenlerde bile, Glutarik asit (glutaric acid) ve 3OH-GA yükselmeleri gösterir.

Glutarik asit (glutaric acid)

Snf Küçük Molekül

LAÇ ETKLEM

Asetazolamid Glutarik asit (glutaric acid) atlm, Asetazolamid ile kombine edildiinde azalabilir.

Asetilsalisilik asit Glutarik asit (glutaric acid) atlm, Asetilsalisilik asit ile kombine edildiinde azalabilir.

Asiklovir Glutarik asit (glutaric acid) atlm, Asiklovir ile kombine edildiinde azalabilir.

Adefovir dipivoksil, Adefovir dipivoksil ile kombine edildiinde Glutarik asit (glutaric acid) atlm azalabilir.

Allopurinol Allopurinolün atlm, Glutarik asit (glutaric acid) ile kombine edildiinde azalabilir.

Alprostadil Alprostadil’in atlm, Glutarik asit (glutaric acid) ile kombine edildiinde azalabilir.

Aminohippurik asit Aminohippurik asit ile kombine edildiinde Glutarik asit (glutaric acid) atlm azalabilir.

Aminofenazon Glutarik asit (glutaric acid)in atlm Aminofenazon ile kombine edildiinde azalabilir.

Amoksisilin Glutarik asit (glutaric acid) atlm, Amoksisilin ile kombine edildiinde azalabilir.

Antipirin Glutarik asit (glutaric acid)in atlm, Antipirin ile kombine edildiinde azaltlabilir.

Bu analitik yöntemin bir snrlamas, birkaç izomer arasnda ayrm yapamamasdr. LC-MS / MS ile 2-hidroksi Glutarik asit (glutaric acid) (2OH-GA), 3-hidroksi Glutarik asit (glutaric acid) (3OH-GA), Glutarik asit (glutaric acid) (GA), metilsüksinik asit (MSA) ve etilmalonik asidin (EMA) ek testi, C4-asilkarnitin ve glutarilkarnitin / C10-OH izomerleri arasnda daha iyi farkllama.

Çoklu açil-CoA dehidrojenaz eksiklii (MADD) olarak da bilinen glutarik asidemi (GA-2), elektron transfer flavoproteinindeki (ETF) veya ETF-ubikinon oksidoredüktazdaki kusurlardan kaynaklanr. Bu hastalk iddetli olabilir ve genellikle yaamn ilk haftalarnda ölümcül olabilir; tipik hipoglisemi semptomlar, kas güçsüzlüü, metabolik asidoz, dismorfik özellikler, kalp kusurlar veya aritmiler, böbrek kistleri ve karacierin ya infiltrasyonu. GA-2, çocuklukta Reye benzeri hastalklar ve çocukluk ve yetikinlikte kas güçsüzlüü ile birlikte etilmalonik-adipik asidüri olarak da bilinen daha hafif bir görünüme sahip olabilir. Glutarik asit (glutaric acid)teki yükselmelere ek olarak, GA-2’li bireyler ayrca artm EMA, MSA ve 2OH-GA gösterebilir.

Referans deerleri

2-OH Glutarik asit (glutaric acid): 3-OH Glutarik asit (glutaric acid): Glutarik asit (glutaric acid): Metilsüksinik asit: Etilmalonik asit: Yüksek C4 balamnda normal EMA seviyeleri, izobutiril-CoA dehidrojenaz (IBDH) eksiklii tehisi ile tutarldr.

Glutarik asit (glutaric acid) (GA) ve 3-hidroksi Glutarik asit (glutaric acid) (3OH-GA) yükselmesi, glutarik asidemi tip 1 (GA-1) tehisi ile tutarldr.

GA, 2-hidroksi Glutarik asit (glutaric acid) (2OH-GA), 3OH-GA, EMA ve MSA’nn yükselmesi, glutarik asidemi (GA-2) tehisi ile tutarldr.

2. Kolker S, Christensen E, Leonar JV, ve dierleri: Glutarik asidüri tip I’in tan ve tedavisi gözden geçirilmi öneriler. J Inherit Metab Dis 2011; 34: 677-694

3. Frerman FE, Goodman SI: Bölüm 103: Elektron transfer flavoprotein ve elektron transfer flavoprotein-ubikinon oksidoredüktaz kusurlar: Glutarik Asidemi Tip II. Scriver’n Kaltsal Hastaln Çevrimiçi Metabolik ve Moleküler Temellerinde. CR Scriver, AL Beaudet, D Valle ve dierleri tarafndan düzenlenmitir. Eriim tarihi 8/17/17. Glutarik Asidüri Tip I’de mevcuttur

1) Glutarik asidüri IIA (GA IIA), glutarikasidüri II’nin neonatal formudur. Bu glutarik asidüri II formu, kanda ve idrarda büyük miktarlarda glutarik ve dier asitlerle karakterize, çok nadir görülen, X’e bal kaltsal bir hastalktr. Bozuklua mitokondride elektron aktaran flavoproteinin disfonksiyonu neden olur.

2) Glutarik asidüri IIB (GA IIB; etilmalonik adipikasidüri), glutarikasidüri II’nin yetikin formudur. Bozukluun bu daha hafif formu, otozomal resesif bir modelde kaltlr. Yetikinlik döneminde vücut dokularnn asiditesi (metabolik asidoz) ve vücut dokularnda yüksek keton seviyesi (ketoz) olmakszn düük kan ekeri seviyesi (hipoglisemi) ortaya çkar. Kandaki ve idrardaki büyük miktarlarda Glutarik asit (glutaric acid), multipl asil-CoA dehidrojenaz enziminin eksikliinden kaynaklanr. (Bu hastalk hakknda daha fazla bilgi için Nadir Hastalk Veritabannda arama teriminiz olarak “glutarik asidüri II” yi seçin.)

Glutarik asidüri III, glutarik asidin birikmesi veya atlmas ile karakterize ve C7ORF10 genindeki mutasyonlarn neden olduu otozomal resesif bir genetik durumdur. Belirtiler deiiklik gösterir ve baz kiiler hiçbir belirti göstermez

Goodman SI, Frerman FE. Lizin oksidasyonundaki kusurlara bal organik asidemiler: 2-ketoadipik asidemi ve glutarik asidemi. In: Scriver CR, Beaudet AL, Sly WS, ve dierleri. Eds. Kaltsal Hastaln Metabolik Moleküler Temeli. 7. bask. McGraw-Hill irketleri. New York, NY; 1995: 1451-60.

3.6. Glutarik Asidin Kaspaz 3 Transkript ve Protein Düzeyleri Üzerindeki Etkisi

Apoptotik yürütücü kaspaz 3’ün mRNA ekspresyonunu izlemek için kantitatif RT-PCR gerçekletirildi (ekil 9 (a)). Karlatrmal yöntem, göreceli ifade seviyelerini analiz etmek için kullanld. 1, 10, 25 ve 50 mM GA ile muameleden 6 saat sonra kaspaz 3 mRNA ekspresyonu, kontrole kyasla srasyla yaklak 1.40 kat, 1.67 kat ve 1.95 kat yukar regüle edildi. Böylece GA, kaspaz 3 aktivasyonu yoluyla apoptozu indükleyebilir.

4. Tartma

Glutarik asidüri tip I, vücut svlarnda yüksek GA, 3-hidroksiGlutarik asit (glutaric acid) (3-OHGA), glutaril-CoA seviyeleri ve ayrca striatal ve frontotemporal kortikal nöronlardaki dejeneratif deiiklikler ile karakterize otozomal resesif bir hastalktr. Serebral GCDH aktivitesinin bir eksiklii, GA I hastalarnda nörolojik hasarn gelimesine atfedilir. Bununla birlikte, bazal gangliyonlardaki dejenerasyon mekanizmasnn anlalmas hala ksmidir.

Glutarik asit (glutaric acid), C3H6 (COOH) 2 formülüne sahip organik bileiktir. lgili “dorusal” dikarboksilik asitler adipik ve süksinik asitler, oda scaklnda sadece yüzde birkaç orannda suda çözünür olmalarna ramen, glutarik asidin suda çözünürlüü% 50’nin (arlk / arlk) üzerindedir.

Glutarik asit (glutaric acid), lizin ve triptofan dahil olmak üzere baz amino asitlerin metabolizmas srasnda vücutta doal olarak üretilir. Bu metabolik yoldaki kusurlar, toksik yan ürünlerin biriktii ve iddetli ensefalopatiye neden olabilen glutarik asidüri ad verilen bir bozuklua yol açabilir.

Glutarik asit (glutaric acid), diasite hidrolize olan kark potasyum karboksilat-nitril vermek üzere butirolaktonun potasyum siyanür ile halka açlmasyla hazrlanabilir. [1] Alternatif olarak hidroliz, ardndan dihidropiran oksidasyonu, Glutarik asit (glutaric acid) verir. Dinitril elde etmek için 1,3-dibromopropann sodyum veya potasyum siyanür ile reaksiyona sokulmas ve ardndan hidroliz yoluyla da hazrlanabilir.

Yaygn bir plastikletirici ve poliester öncüsü olan 1,5-Pentandiol, Glutarik asit (glutaric acid) ve türevlerinin hidrojenasyonu ile üretilir. [2]

Polyester polioller, poliamidler gibi polimerlerin üretiminde glutarik asidin kendisi kullanlmtr. Tek sayda karbon atomu (yani 5), polimer esnekliini azaltmada yararldr. [Kaynak belirtilmeli]

Uvitonik asit, amonyan Glutarik asit (glutaric acid) üzerindeki etkisiyle elde edilir.

Glutarik asit (glutaric acid) ciltte ve gözlerde tahrie neden olabilir. [3] Akut tehlikeler, bu bileiin yutulmas, solunmas veya cilt tarafndan emilmesi yoluyla zararl olabilecei gerçeini içerir. [3]

Glutarik asit (glutaric acid) (Pentandioik Asit) dorusal bir dikarboksilik asittir. Siklopentan, siklopentanol ve siklopentanon oksitlenerek hazrlanmtr. [9]

Glutarik asit (glutaric acid) uygulamas

Glutarik asit (glutaric acid), glutarik anhidrit sentezinde balangç reaktifi olarak kullanlabilir. [9]

Kronik olarak yüksek Glutarik asit (glutaric acid) seviyeleri, glutarik asidüri tip I dahil olmak üzere en az üç doutan metabolizma hatasyla ilikilidir.

Glutarik asit (glutaric acid), C3H6 (COOH) 2 formülüne sahip organik bileiktir. lgili “dorusal” dikarboksilik asitler adipik ve süksinik asitler, oda scaklnda sadece yüzde birkaç orannda suda çözünebilmesine ramen, glutarik asidin suda çözünürlüü% 50’nin (arlk / arlk) üzerindedir.

Glutarik asit (glutaric acid), dikarboksilik asitler arasnda en düük erime noktasna sahiptir (98 C); suda çok çözünür ve sudaki çözelti orta kuvvetli bir asittir. Glutarik aside ksa süreli maruz kalma, gözleri, cildi ve solunum yollarn tahri edebilir.

GLUTARIC ACID (GLUTARK AST)

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