2-Methylcitric acid

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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
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Contents

Introduction

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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)

Biological Function

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Catabolism

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Diseases / Conditions Related to Nutrition

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Other (Monogenic) Disorders

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Nutritional Information

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Drivers for biological variation

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Vulnerable groups

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