InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: OXCT1

Summary

InnateDB Protein

IDBP-18721.6

Last Modified

2014-10-13 [Report errors or provide feedback]

Gene Symbol

OXCT1

Protein Name

3-oxoacid CoA transferase 1

Synonyms

OXCT; SCOT;

Species

Homo sapiens

Ensembl Protein

ENSP00000196371

InnateDB Gene

IDBG-18717 (OXCT1)

Protein Structure

UniProt Annotation

Function

Key enzyme for ketone body catabolism. Transfers the CoA moiety from succinate to acetoacetate. Formation of the enzyme-CoA intermediate proceeds via an unstable anhydride species formed between the carboxylate groups of the enzyme and substrate.

Subcellular Localization

Mitochondrion matrix.

Disease Associations

Succinyl-CoA-3-ketoacid-CoA transferase deficiency (SCOTD) [MIM:245050]: A disorder of ketone body metabolism, characterized by episodic ketoacidosis. Patients are usually asymptomatic between episodes. {ECO:0000269PubMed:10964512, ECO:0000269PubMed:21296660, ECO:0000269PubMed:9671268}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Abundant in heart, followed in order by kidney, brain, and muscle, whereas in liver it is undetectable; also detectable in leukocytes and fibroblasts.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 45 experimentally validated interaction(s) in this database.
They are also associated with 1 interaction(s) predicted by orthology.

Experimentally validated
Total	45 [view]
Protein-Protein	45 [view]
Protein-DNA	0
Protein-RNA	0
DNA-DNA	0
RNA-RNA	0
DNA-RNA	0

Predicted by orthology
Total	1 [view]

Gene Ontology

Molecular Function

Accession	GO Term
GO:0008260	3-oxoacid CoA-transferase activity
GO:0008410	CoA-transferase activity
GO:0042803	protein homodimerization activity

Biological Process

GO:0007420	brain development
GO:0007507	heart development
GO:0007584	response to nutrient
GO:0008152	metabolic process
GO:0009725	response to hormone
GO:0014823	response to activity
GO:0035774	positive regulation of insulin secretion involved in cellular response to glucose stimulus
GO:0042182	ketone catabolic process
GO:0042493	response to drug
GO:0042594	response to starvation
GO:0044255	cellular lipid metabolic process
GO:0044281	small molecule metabolic process
GO:0045471	response to ethanol
GO:0046950	cellular ketone body metabolic process
GO:0046952	ketone body catabolic process
GO:0060612	adipose tissue development

Cellular Component

GO:0005739	mitochondrion
GO:0005759	mitochondrial matrix

Protein Structure and Domains

PDB ID

InterPro

IPR004165 Coenzyme A transferase family I
IPR012791 3-oxoacid CoA-transferase, subunit B
IPR012792 3-oxoacid CoA-transferase, subunit A
IPR014388 3-oxoacid CoA-transferase

PFAM

PF01144

PRINTS

PIRSF

PIRSF000858

SMART

SM00882

TIGRFAMs

Post-translational Modifications

Modification

Cross-References

SwissProt