InnateDB: Systems Biology of the Innate Immune Response

Homo sapiens Protein: PRKRA

Summary

InnateDB Protein

IDBP-76032.6

Last Modified

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

Gene Symbol

PRKRA

Protein Name

protein kinase, interferon-inducible double stranded RNA dependent activator

Synonyms

DYT16; PACT; RAX;

Species

Homo sapiens

Ensembl Protein

ENSP00000318176

InnateDB Gene

IDBG-76030 (PRKRA)

Protein Structure

UniProt Annotation

Function

Activates EIF2AK2/PKR in the absence of double-stranded RNA (dsRNA), leading to phosphorylation of EIF2S1/EFI2-alpha and inhibition of translation and induction of apoptosis. Required for siRNA production by DICER1 and for subsequent siRNA-mediated post- transcriptional gene silencing. Does not seem to be required for processing of pre-miRNA to miRNA by DICER1. Promotes UBC9-p53/TP53 association and sumoylation and phosphorylation of p53/TP53 at 'Lys-386' at 'Ser-392' respectively and enhances its activity in a EIF2AK2/PKR-dependent manner (By similarity). {ECO:0000250}.

Subcellular Localization

Cytoplasm, perinuclear region. Cytoplasm.

Disease Associations

Dystonia 16 (DYT16) [MIM:612067]: An early-onset dystonia-parkinsonism disorder. Dystonia is defined by the presence of sustained involuntary muscle contraction, often leading to abnormal postures. DYT16 patients have progressive, generalized dystonia with axial muscle involvement, oro-mandibular (sardonic smile) and laryngeal dystonia and, in some cases, parkinsonian features. {ECO:0000269PubMed:18243799, ECO:0000269PubMed:18420150}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Tissue Specificity

Comments

Interactions

Number of Interactions

This gene and/or its encoded proteins are associated with 73 experimentally validated interaction(s) in this database.

Experimentally validated
Total	73 [view]
Protein-Protein	69 [view]
Protein-DNA	3 [view]
Protein-RNA	0
DNA-DNA	1 [view]
RNA-RNA	0
DNA-RNA	0

Gene Ontology

Molecular Function

Accession	GO Term
GO:0005515	protein binding
GO:0008047	enzyme activator activity
GO:0016301	kinase activity
GO:0042803	protein homodimerization activity
GO:0044822	poly(A) RNA binding

Biological Process

GO:0006468	protein phosphorylation
GO:0006955	immune response
GO:0008285	negative regulation of cell proliferation
GO:0009615	response to virus
GO:0010467	gene expression
GO:0030422	production of siRNA involved in RNA interference
GO:0034599	cellular response to oxidative stress
GO:0042473	outer ear morphogenesis
GO:0042474	middle ear morphogenesis
GO:0043085	positive regulation of catalytic activity
GO:0045087	innate immune response (InnateDB)
GO:0048705	skeletal system morphogenesis
GO:2001244	positive regulation of intrinsic apoptotic signaling pathway