74626
(E)-2-Methylglutaconic acid
≥98.0% (HPLC)
Synonym(s):
(2E)-2-Methyl-2-pentenedioic acid, trans-2-Methylglutaconic acid
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About This Item
Empirical Formula (Hill Notation):
C6H8O4
CAS Number:
Molecular Weight:
144.13
NACRES:
NA.24
UNSPSC Code:
41116107
Beilstein/REAXYS Number:
2096229
Quality Level
assay
≥98.0% (HPLC)
mp
141-147 °C
application(s)
clinical testing
format
neat
storage temp.
2-8°C
InChI
1S/C6H8O4/c1-4(6(9)10)2-3-5(7)8/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+
InChI key
JKGHDBJDBRBRNA-DUXPYHPUSA-N
Biochem/physiol Actions
2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase.
signalword
Warning
hcodes
pcodes
Hazard Classifications
Skin Sens. 1
Storage Class
13 - Non Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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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
Solution of a classical problem. Tautomerism and isomerism in the α-methylglutaconic acid series
Kagan, J., et al.
The Journal of Organic Chemistry, 40, 3085-3093 (1975)
M Fontaine et al.
Clinica chimica acta; international journal of clinical chemistry, 255(1), 67-83 (1996-11-15)
We report clinical and biological investigations in two patients (twin brothers) with 2-methylacetoacetyl-CoA thiolase deficiency. Main clinical features included important staturo-ponderal delay, frequent infectious rhinopharyngitis episodes and an acute metabolic acidosis at the age of 4 years, this metabolic decompensation
Global Trade Item Number
| SKU | GTIN |
|---|---|
| 74626-50MG | 04061833415900 |
| 74626-10MG | 04061833388259 |