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06689

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3-Methylglutaconic acid, mixture of E and Z isomers

≥98.0% (HPLC)

Synonyme(s) :

3-Methyl-2-pentenedioic acid, NSC 249232

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About This Item

Formule empirique (notation de Hill):
C6H8O4
Numéro CAS:
5746-90-7
Poids moléculaire :
144.13
Numéro Beilstein :
1722907
Numéro MDL:
MFCD01556044
Code UNSPSC :
41116107
ID de substance PubChem :
329748259
Nomenclature NACRES :
NA.24

Niveau de qualité

100

Pureté

≥98.0% (HPLC)

Application(s)

clinical testing

Format

neat

Température de stockage

2-8°C

Chaîne SMILES 

C/C(CC(O)=O)=C\C(O)=O

InChI

1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+

Clé InChI

WKRBKYFIJPGYQC-DUXPYHPUSA-N

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Catégories apparentées

Actions biochimiques/physiologiques

3-Methylglutaconic acid is a metabolite (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. 3-Methylglutaconic acid accumulates in patients with a deficiency of 3-methylglutaconyl-CoA hydratase.

Code de la classe de stockage

11 - Combustible Solids

Classe de danger pour l'eau (WGK)

WGK 3

Point d'éclair (°F)

Not applicable

Point d'éclair (°C)

Not applicable

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Certificats d'analyse (COA)

Lot/Batch Number
BCBN6472
BCBN6472V
BCBN6472

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G A Mitchell et al.
Prenatal diagnosis, 15(8), 725-729 (1995-08-01)
We report the first molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase (HL) deficiency. The proband had a classic but severe presentation with hypoketotic hypoglycaemia and acidosis, secondary mental retardation, and epilepsy, and HL deficiency was documented in cultured fibroblasts. We
E Holme et al.
Pediatric research, 32(6), 731-735 (1992-12-01)
We report the finding of mitochondrial ATP-synthase deficiency in a child with persistent 3-methylglutaconic aciduria. The child presented in the neonatal period with severe lactic acidosis, which was controlled by Na-HCO3 and glucose infusions. During the 1st y of life
R I Kelley et al.
Pediatric research, 37(5), 671-674 (1995-05-01)
The branched-chain organic acid, 3-methylglutaconic acid, is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. Because the majority of patients with
R I Kelley
Clinica chimica acta; international journal of clinical chemistry, 220(2), 157-164 (1993-11-15)
A method is described for quantification of the trace metabolite, 3-methylglutaconic acid, by isotope-dilution gas chromatography/mass spectrometry using synthetic 3-[2,4,6-13C3]methylglutaconic acid. Results are shown for quantification of 3-methylglutaconic acid in plasma, urine, cerebrospinal fluid and amniotic fluid for both normal
H Costeff et al.
Annals of neurology, 33(1), 103-104 (1993-01-01)
Behr's syndrome consists of recessively inherited infantile optic atrophy, together with chronic neurological disturbances such as ataxia, extrapyramidal dysfunction, and juvenile spastic paresis. This syndrome was found to be relatively common among Iraqi Jews. For our study, 18 such patients
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