42641
(Z)-3-Methylglutaconic acid
≥97.0% (HPLC)
Sinónimos:
(2Z)-3-Methyl-2-pentenedioic acid
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About This Item
Fórmula empírica (notación de Hill):
C6H8O4
Número de CAS:
Peso molecular:
144.13
Beilstein/REAXYS Number:
1722908
UNSPSC Code:
41116107
NACRES:
NA.24
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Quality Level
assay
≥97.0% (HPLC)
mp
147-151 °C
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
2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase.
Storage Class
11 - Combustible Solids
wgk_germany
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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Brian J Wong et al.
Biochemistry, 43(16), 4646-4654 (2004-04-21)
Members of the enoyl-CoA hydratase (crotonase) superfamily catalyze different overall reactions that utilize a common catalytic strategy delivered by a shared structural scaffold; the substrates are usually acyl esters of coenzyme A, and the intermediates are usually thioester enolate anions
W Meier-Augenstein et al.
Journal of chromatography, 615(1), 127-135 (1993-05-19)
An improved method for the identification and quantification of organic acids in body fluids employing capillary gas chromatography-mass spectrometry has been developed. A thick-film capillary column, that combines the properties of a capillary column with those of a megabore column
Utilization of branched chain acids in cholesterol synthesis.
K BLOCH et al.
The Journal of biological chemistry, 211(2), 687-699 (1954-12-01)
Rebecca Young et al.
Biochemical and biophysical research communications, 534, 261-265 (2020-12-08)
3-methylglutaconic (3MGC) aciduria is associated with a growing number of discrete inborn errors of metabolism. Herein, an antibody-based approach to detection/quantitation of 3MGC acid has been pursued. When trans-3MGC acid conjugated keyhole limpet hemocyanin (KLH) was inoculated into rabbits a
M Duran et al.
Biomedical mass spectrometry, 9(1), 1-5 (1982-01-01)
The identification of (E)-2-methylglutaconic acid, a 'new' metabolite of isoleucine, is described. The substance was detected in urine samples from patients with propionic acidaemia, methylmalonic acidaemia and so-called beta-ketothiolase deficiency; in the majority of cases together with N-tiglylglycine. (E)-2-Methylglutaconic acid
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