06689
3-Methylglutaconic acid, mixture of E and Z isomers
≥98.0% (HPLC)
Synonym(s):
3-Methyl-2-pentenedioic acid, NSC 249232
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About This Item
Empirical Formula (Hill Notation):
C6H8O4
CAS Number:
Molecular Weight:
144.13
UNSPSC Code:
41116107
NACRES:
NA.24
PubChem Substance ID:
MDL number:
Beilstein/REAXYS Number:
1722907
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
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.
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Storage Class
11 - Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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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