06689
3-Methylglutaconic acid, mixture of E and Z isomers
≥98.0% (HPLC)
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Synonym(s):
3-Methyl-2-pentenedioic acid, NSC 249232
Empirical Formula (Hill Notation):
C6H8O4
CAS Number:
Molecular Weight:
144.13
Beilstein:
1722907
MDL number:
UNSPSC Code:
41116107
PubChem Substance ID:
NACRES:
NA.24
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Quality Level
Assay
≥98.0% (HPLC)
application(s)
clinical testing
format
neat
storage temp.
2-8°C
SMILES string
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+
InChI key
WKRBKYFIJPGYQC-DUXPYHPUSA-N
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Biochem/physiol Actions
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.
WGK
WGK 3
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
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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
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
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
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