Accession Number | HMDB03520 |
Common_Name | 2-Methylglutaconic acid |
Description | 2-Methylglutaconic acid is found in the urine of patients with organic aciduria from 2-methylacetoacetyl-CoA thiolase (EC 2.3.1.9, acetyl-CoA C-acetyltransferase) deficiency (ACAT, OMIM 607809). Main clinical features of ACAT include important staturo-ponderal delay, frequent infectious rhinopharyngitis episodes and an acute metabolic acidosis (this metabolic decompensation being adequately halted by bicarbonate supplementation). (PMID: 8930414, 2925825) |
Chemical_IUPAC_Name | 2-methylpent-2-enedioic acid |
Chemical Formula | C6H8O4 |
Sample Concentration | Not Available |
Mass Value | Not Available |
Mass Unit | Not Available |
Manufacturer | Not Available |
Analyzer | Not Available |
Delivery | Not Available |
Ionization | Not Available |
Predicted 1H NMR Spectrum | Download |
Predicted 1H NMR Peaklist | Download |
Predicted 13C NMR Spectrum | Download |
Predicted 13C NMR Peaklist | Download |
Sample Concentration | Not Available |
Mass Value | Not Available |
Mass Unit | Not Available |
Manufacturer | Not Available |
Frequency | Not Available |
1H NMR Spectrum | Not Available |
Sample Concentration | Not Available |
Mass Value | Not Available |
Mass Unit | Not Available |
Manufacturer | Not Available |
Frequency | Not Available |
13C NMR Spectrum | Not Available |
Low Energy Voltage | Not Available |
Low Energy Spectrum | Not Available |
Low Energy Peaklist | Not Available |
Medium Energy Spectrum | Not Available |
Hight Energy Spectrum | Not Available |