Go Annotations as Summary Text (Tabular View) (GO Graph)

GO curators for mouse genes have assigned the following annotations to the gene product of Hadha. (This text reflects annotations as of Wednesday, January 23, 2013.) MGI curation of this mouse gene is considered complete, including annotations derived from the biomedical literature as of May 5, 2011. If you know of any additional information regarding this mouse gene please let us know. Please supply mouse gene symbol and a PubMed ID.

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Summary from NCBI RefSeq

[Summary is not available for the mouse gene. This summary is for the human ortholog.] This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. [provided by RefSeq, Jul 2008]

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Summary text based on GO annotations supported by experimental evidence in mouse

• Researchers have inferred from direct assay, that the gene product of Hadha
  • participates in the following biological processes:
    • oxidation-reduction process ^[6]
  • performs the following molecular functions:
    • long-chain-3-hydroxyacyl-CoA dehydrogenase activity ^[6]
  • is located in the following cellular components:
    • mitochondrial inner membrane ^[1]
    • mitochondrion ^{[4, 5]}
• Researchers have inferred, based on phenotypic analysis of mouse mutants, that the gene product of Hadha
  • participates in the following biological processes:
    • fatty acid beta-oxidation ^[3]
    • response to insulin stimulus ^[2]

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Summary text based on GO annotations supported by experimental evidence in other organisms

• From comparative sequence analysis (see table for details), MGI curators have determined that the gene product of Hadha:
  • participates in the following biological processes:
    • fatty acid beta-oxidation
    • response to drug
  • performs the following molecular functions:
    • 3-hydroxyacyl-CoA dehydrogenase activity
    • acetyl-CoA C-acyltransferase activity
    • enoyl-CoA hydratase activity
    • fatty-acyl-CoA binding
    • long-chain-3-hydroxyacyl-CoA dehydrogenase activity
    • long-chain-enoyl-CoA hydratase activity
    • NAD binding
    • protein complex binding
  • is located in the following cellular components:
    • cytoplasm
    • mitochondrial fatty acid beta-oxidation multienzyme complex
    • mitochondrial nucleoid
    • mitochondrion
    • nucleolus

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Summary text based on GO annotations supported by structural data

• Hadha encodes a protein or proteins that contain the following InterPro domains: 3-hydroxyacyl-CoA dehydrogenase, C-terminal, 3-hydroxyacyl-CoA dehydrogenase, conserved site, 3-hydroxyacyl-CoA dehydrogenase, NAD binding, 6-phosphogluconate dehydrogenase, C-terminal-like, Crotonase, core, Dehydrogenase, multihelical, Enoyl-CoA hydratase/isomerase, conserved site, Fatty acid oxidation complex, alpha subunit, mitochondrial, NAD(P)-binding domain, indicating that the gene product:
  • participates in the following biological processes:
    • fatty acid metabolic process
  • performs the following molecular functions:
    • catalytic activity
    • coenzyme binding
    • nucleotide binding
    • oxidoreductase activity
    • oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor

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Summary text for additional MGI annotations

• Automated translation to GO terms of SwissProt keywords that describe Hadha indicates that it:
  • participates in the following biological processes:
    • fatty acid metabolic process
    • lipid metabolic process
  • performs the following molecular functions:
    • catalytic activity
    • lyase activity
    • oxidoreductase activity

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References

1. Da Cruz S et al. (2003) Proteomic analysis of the mouse liver mitochondrial inner membrane. J Biol Chem, 278:41566-71. (PubMed:12865426)
2. Ibdah JA et al. (2005) Mice heterozygous for a defect in mitochondrial trifunctional protein develop hepatic steatosis and insulin resistance. Gastroenterology, 128:1381-90. (PubMed:15887119)
3. Ibdah JA et al. (2001) Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death. J Clin Invest, 107:1403-9. (PubMed:11390422)
4. Mootha VK et al. (2003) Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria. Cell, 115:629-40. (PubMed:14651853)
5. Pagliarini DJ et al. (2008) A mitochondrial protein compendium elucidates complex I disease biology. Cell, 134:112-23. (PubMed:18614015)
6. Shekhawat PS et al. (2004) Carnitine content and expression of mitochondrial beta-oxidation enzymes in placentas of wild-type (OCTN2(+/+)) and OCTN2 Null (OCTN2(-/-)) Mice. Pediatr Res, 56:323-8. (PubMed:15240869)

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Gene Ontology Evidence Code Abbreviations:

EXP Inferred from experiment

IC Inferred by curator

IDA Inferred from direct assay

IEA Inferred from electronic annotation

IGI Inferred from genetic interaction

IMP Inferred from mutant phenotype

IPI Inferred from physical interaction

ISS Inferred from sequence or structural similarity

ISO Inferred from sequence orthology

ISA Inferred from sequence alignment

ISM Inferred from sequence model

NAS Non-traceable author statement

ND No biological data available

RCA Reviewed computational analysis

TAS Traceable author statement

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