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品質等級
化驗
≥98.0% (HPLC)
mp
141-147 °C
應用
clinical testing
形式
neat
儲存溫度
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 密鑰
JKGHDBJDBRBRNA-DUXPYHPUSA-N
生化/生理作用
2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase.
訊號詞
Warning
危險聲明
危險分類
Skin Sens. 1
儲存類別代碼
13 - Non Combustible Solids
水污染物質分類(WGK)
WGK 3
閃點(°F)
Not applicable
閃點(°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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