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Merck

74626

Supelco

(E)-2-Methyl-glutaconsäure

≥98.0% (HPLC)

Synonym(e):

(2E)-2-Methyl-2-pentendisäure, trans-2-Methyl-glutaconsäure

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About This Item

Empirische Formel (Hill-System):
C6H8O4
CAS-Nummer:
Molekulargewicht:
144.13
Beilstein:
2096229
UNSPSC-Code:
41116107
NACRES:
NA.24

Qualitätsniveau

Assay

≥98.0% (HPLC)

mp (Schmelzpunkt)

141-147 °C

Anwendung(en)

clinical testing

Format

neat

Lagertemp.

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+

InChIKey

JKGHDBJDBRBRNA-DUXPYHPUSA-N

Biochem./physiol. Wirkung

2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase.

Piktogramme

Exclamation mark

Signalwort

Warning

H-Sätze

Gefahreneinstufungen

Skin Sens. 1

Lagerklassenschlüssel

13 - Non Combustible Solids

WGK

WGK 3

Flammpunkt (°F)

Not applicable

Flammpunkt (°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
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
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

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