Identification of FAH domain-containing protein 1 (FAHD1) as oxaloacetate decarboxylase

J Biol Chem. 2015 Mar 13;290(11):6755-62. doi: 10.1074/jbc.M114.609305. Epub 2015 Jan 9.

Abstract

Fumarylacetoacetate hydrolase (FAH) domain-containing proteins occur in both prokaryotes and eukaryotes, where they carry out diverse enzymatic reactions, probably related to structural differences in their respective FAH domains; however, the precise relationship between structure of the FAH domain and the associated enzyme function remains elusive. In mammals, three FAH domain-containing proteins, FAHD1, FAHD2A, and FAHD2B, are known; however, their enzymatic function, if any, remains to be demonstrated. In bacteria, oxaloacetate is subject to enzymatic decarboxylation; however, oxaloacetate decarboxylases (ODx) were so far not identified in eukaryotes. Based on molecular modeling and subsequent biochemical investigations, we identified FAHD1 as a eukaryotic ODx enzyme. The results presented here indicate that dedicated oxaloacetate decarboxylases exist in eukaryotes.

Keywords: Computer Modeling; Decarboxylase; Energy Metabolism; FAH Domain; FAHD1; Mitochondria; Oxaloacetate Decarboxylase; Pyruvate.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Sequence
  • Animals
  • Carboxy-Lyases / chemistry
  • Carboxy-Lyases / genetics
  • Carboxy-Lyases / metabolism*
  • Crystallography, X-Ray
  • Energy Metabolism
  • Female
  • Gene Expression Regulation
  • Humans
  • Hydrolases / chemistry
  • Hydrolases / genetics
  • Hydrolases / metabolism*
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Models, Molecular
  • Molecular Sequence Data
  • Protein Conformation
  • Protein Structure, Tertiary
  • Pyruvic Acid / metabolism
  • Sequence Alignment

Substances

  • Pyruvic Acid
  • Hydrolases
  • fumarylacetoacetase
  • Carboxy-Lyases
  • oxaloacetate decarboxylase