Aliases for HSPH1 Gene
External Ids for HSPH1 Gene
Previous GeneCards Identifiers for HSPH1 Gene
This gene encodes a member of the heat shock protein 70 family of proteins. The encoded protein functions as a nucleotide exchange factor for the molecular chaperone heat shock cognate 71 kDa protein (Hsc70). In addition, this protein plays a distinct but related role as a holdase that inhibits the aggregation of misfolded proteins, including the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Elevated expression of this protein has been observed in numerous human cancers. [provided by RefSeq, Mar 2017]
GeneCards Summary for HSPH1 Gene
HSPH1 (Heat Shock Protein Family H (Hsp110) Member 1) is a Protein Coding gene. Diseases associated with HSPH1 include Cystic Fibrosis. Among its related pathways are Protein processing in endoplasmic reticulum and Cellular response to heat stress. Gene Ontology (GO) annotations related to this gene include alpha-tubulin binding. An important paralog of this gene is HSPA4.
UniProtKB/Swiss-Prot for HSPH1 Gene
Acts as a nucleotide-exchange factor (NEF) for chaperone proteins HSPA1A and HSPA1B, promoting the release of ADP from HSPA1A/B thereby triggering client/substrate protein release (PubMed:24318877). Prevents the aggregation of denatured proteins in cells under severe stress, on which the ATP levels decrease markedly. Inhibits HSPA8/HSC70 ATPase and chaperone activities (By similarity).