InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: COASY

Summary

InnateDB Protein

IDBP-242849.6

Last Modified

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

Gene Symbol

COASY

Protein Name

CoA synthase

Synonyms

Species

Homo sapiens

Ensembl Protein

ENSP00000377406

InnateDB Gene

IDBG-51077 (COASY)

Protein Structure

UniProt Annotation

Function

Bifunctional enzyme that catalyzes the fourth and fifth sequential steps of CoA biosynthetic pathway. The fourth reaction is catalyzed by the phosphopantetheine adenylyltransferase, coded by the coaD domain; the fifth reaction is catalyzed by the dephospho-CoA kinase, coded by the coaE domain. May act as a point of CoA biosynthesis regulation. {ECO:0000269PubMed:11923312}.

Subcellular Localization

Cytoplasm {ECO:0000250}. Mitochondrion matrix {ECO:0000269PubMed:24360804}. Note=The protein is mainly present in the mitochondrial matrix, probably anchored to the inner mitochondrial membrane, but is also present in cell lysate.

Disease Associations

Neurodegeneration with brain iron accumulation 6 (NBIA6) [MIM:615643]: A neurodegenerative disorder associated with iron accumulation in the brain, primarily in the basal ganglia. It is characterized by progressive motor and cognitive dysfunction beginning in childhood or young adulthood. Patients show extrapyramidal motor signs, such as spasticity, dystonia, and parkinsonism. {ECO:0000269PubMed:24360804}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Expressed in all tissues examined including brain, heart, skeletal muscle, colon, thymus, spleen, kidney, liver, small intestine, placenta, lung and peripheral blood leukocyte. Lowest expression in peripheral blood leukocytes and highest in kidney and liver. Isoform 2 is expressed mainly in the brain. {ECO:0000269PubMed:11923312}.

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 17 experimentally validated interaction(s) in this database.

Experimentally validated
Total	17 [view]
Protein-Protein	15 [view]
Protein-DNA	1 [view]
Protein-RNA	0
DNA-DNA	1 [view]
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0003824	catalytic activity
GO:0004140	dephospho-CoA kinase activity
GO:0004595	pantetheine-phosphate adenylyltransferase activity
GO:0005515	protein binding
GO:0005524	ATP binding

Biological Process

GO:0006766	vitamin metabolic process
GO:0006767	water-soluble vitamin metabolic process
GO:0008219	cell death
GO:0009058	biosynthetic process
GO:0009108	coenzyme biosynthetic process
GO:0015937	coenzyme A biosynthetic process
GO:0015939	pantothenate metabolic process
GO:0016310	phosphorylation
GO:0044281	small molecule metabolic process