DNAJA3

This gene encodes a member of the DNAJ/Hsp40 protein family. DNAJ/Hsp40 proteins stimulate the ATPase activity of Hsp70 chaperones and play critical roles in protein folding, degradation, and multimeric complex assembly. The encoded protein is localized to mitochondria and mediates several cellular processes including proliferation, survival and apoptotic signal transduction. The encoded protein also plays a critical role in tumor suppression through interactions with oncogenic proteins including ErbB2 and the p53 tumor suppressor protein. Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene. [provided by RefSeq, Aug 2011]

Full Name

DnaJ Heat Shock Protein Family (Hsp40) Member A3

Function

Modulates apoptotic signal transduction or effector structures within the mitochondrial matrix. Affect cytochrome C release from the mitochondria and caspase 3 activation, but not caspase 8 activation. Isoform 1 increases apoptosis triggered by both TNF and the DNA-damaging agent mytomycin C; in sharp contrast, isoform 2 suppresses apoptosis. Can modulate IFN-gamma-mediated transcriptional activity. Isoform 2 may play a role in neuromuscular junction development as an effector of the MUSK signaling pathway.

Biological Process

Activation-induced cell death of T cells Source: Ensembl
Activation of cysteine-type endopeptidase activity involved in apoptotic process Source: UniProtKB
Cell aging Source: Ensembl
Mitochondrial DNA replication Source: Ensembl
Mitochondrion organization Source: GO_Central
Negative regulation of apoptotic process Source: UniProtKB
Negative regulation of cell population proliferation Source: UniProtKB
Negative regulation of cysteine-type endopeptidase activity involved in apoptotic process Source: UniProtKB
Negative regulation of I-kappaB kinase/NF-kappaB signaling Source: UniProtKB
Negative regulation of interferon-gamma-mediated signaling pathway Source: UniProtKB
Negative regulation of NF-kappaB transcription factor activity Source: UniProtKB
Negative regulation of programmed cell death Source: Ensembl
Negative regulation of protein kinase activity Source: UniProtKB
Negative regulation of transcription by RNA polymerase II Source: UniProtKB
Neuromuscular junction development Source: UniProtKB
Positive regulation of apoptotic process Source: UniProtKB
Positive regulation of protein ubiquitination Source: UniProtKB
Positive regulation of T cell proliferation Source: Ensembl
Protein folding Source: UniProtKB
Protein stabilization Source: UniProtKB
Regulation of apoptotic process Source: GO_Central
Response to heat Source: InterPro
Response to interferon-gamma Source: UniProtKB
Skeletal muscle acetylcholine-gated channel clustering Source: UniProtKB
Small GTPase mediated signal transduction Source: Ensembl
T cell differentiation in thymus Source: Ensembl

Cellular Location

Mitochondrion matrix; Cytosol; Postsynaptic cell membrane. Recruited to the postsynaptic cell membrane of the neuromuscular junction through interaction with MUSK.

PTM

Tyrosine phosphorylated.