Aliases for HSPA1L Gene
External Ids for HSPA1L Gene
Previous GeneCards Identifiers for HSPA1L Gene
This gene encodes a 70kDa heat shock protein. In conjunction with other heat shock proteins, this protein stabilizes existing proteins against aggregation and mediates the folding of newly translated proteins in the cytosol and in organelles. The gene is located in the major histocompatibility complex class III region, in a cluster with two closely related genes which also encode isoforms of the 70kDa heat shock protein. [provided by RefSeq, Jul 2008]
GeneCards Summary for HSPA1L Gene
HSPA1L (Heat Shock Protein Family A (Hsp70) Member 1 Like) is a Protein Coding gene. Diseases associated with HSPA1L include Inflammatory Bowel Disease 1 and Paranoid Schizophrenia. Among its related pathways are Regulation of degradation of deltaF508 CFTR in CF and HSF1-dependent transactivation. Gene Ontology (GO) annotations related to this gene include unfolded protein binding. An important paralog of this gene is HSPA1A.
UniProtKB/Swiss-Prot Summary for HSPA1L Gene
Molecular chaperone implicated in a wide variety of cellular processes, including protection of the proteome from stress, folding and transport of newly synthesized polypeptides, activation of proteolysis of misfolded proteins and the formation and dissociation of protein complexes. Plays a pivotal role in the protein quality control system, ensuring the correct folding of proteins, the re-folding of misfolded proteins and controlling the targeting of proteins for subsequent degradation. This is achieved through cycles of ATP binding, ATP hydrolysis and ADP release, mediated by co-chaperones. The affinity for polypeptides is regulated by its nucleotide bound state. In the ATP-bound form, it has a low affinity for substrate proteins. However, upon hydrolysis of the ATP to ADP, it undergoes a conformational change that increases its affinity for substrate proteins. It goes through repeated cycles of ATP hydrolysis and nucleotide exchange, which permits cycles of substrate binding and release (PubMed:26865365). Positive regulator of PRKN translocation to damaged mitochondria (PubMed:24270810).