2-Methylcitric acid

From NuGOwiki

The NuGOwiki Metabolite Database is a joint initiative of NuGO and HMDB

Taxonomy Navigation Box; search by

2-Methylcitric acid
2D structure for 2-Methylcitric acid
Chemical Name	2-hydroxybutane-1,2,3-tricarboxylic acid
Chemical Formula	C₇H₁₀O₇
CAS Number	6061-96-7
Chemical Information	HMDB00379
Biochemical Taxonomy	Tricarboxylic Acids
Functional Taxonomy	Not Available
Nutritional Taxonomy	Not Available
Metabolic Pathways	Not Available
Biofluid Location	Blood Urine
Tissue Location	Not Available
Normal Biofluid Concentrations	Blood: 0.1 (0.08-0.26) umol/L Urine: 2.6 (1.6-6.2) umol/mmol creatinine Urine: 3.2 (2.0-8.1) umol/mmol creatinine Urine: 3.4 (1.4-8.5) umol/mmol creatinine Urine: 6.7 (0.7-19.6) umol/mmol creatinine Urine: 7.7 (3.3-28.6) umol/mmol creatinine
Normal Tissue Concentrations	Not Available
Diseases / Conditions Related to Nutrition	Not Available
Other (Monogenic Disorders)	Not Available
Abnormal Biofluid Concentrations	Not Available
Abnormal Tissue Concentrations	Not Available
Physiological Processes	Not Available

Authors:
Affiliations:

Introduction

guidelines
Methylcitric acid (MCA) is elevated in body fluids of patients with propionic acidaemia (PA; OMIM 232000, 232050), methylmalonic aciduria (MMA; OMIM 251000, 251120) and multiple carboxylase deficiency (OMIM 253260, 253270). MCA is formed by condensation of accumulated propionyl- CoA and oxalacetate by the enzyme si-citrate synthase (EC 4.1.3.7). MCA molecule has two stereogenic centers so that it can occur in the form of four stereoisomers. Only two stereoisomers of MCA, (2S, 3S) and (2R, 3S), were found in human urine. (PMID: 17295121)