75606
(Z)-2-Methylglutaconic acid
≥97.0% (HPLC)
别名:
(Z)-2-Methyl-2-pentenedioic acid
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关于此项目
经验公式（希尔记法）:
C6H8O4
化学文摘社编号:
分子量:
144.13
UNSPSC Code:
12352200
PubChem Substance ID:
MDL number:
InChI
1S/C6H8O4/c1-4(6(9)10)2-3-5(7)8/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2-
SMILES string
OC(C/C=C(C)\C(O)=O)=O
InChI key
JKGHDBJDBRBRNA-RQOWECAXSA-N
assay
≥97.0% (HPLC)
mp
123-126 °C
format
neat
storage temp.
2-8°C
Quality Level
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
存储类别
13 - Non Combustible Solids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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)
Metabolic profiling of urinary organic acids by single and multicolumn capillary gas chromatography.
M F Lefevere et al.
Journal of chromatographic science, 27(1), 23-29 (1989-01-01)
High-resolution gas chromatography (HRGC) and gas chromatography/mass spectrometry (GC/MS) are the techniques of choice to determine the retention indices of more than 200 organic acids as their trimethylsilyl (TMS) or oxime-trimethylsilyl derivatives. Several types of apolar and semipolar fused-silica capillary
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
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