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60 kD heat shock protein, mitochondrial; heat shock protein 60; HSP-60; Hsp60; 60 kD chaperonin; chaperonin 60; CPN60; mitochondrial matrix protein P1; P60 lymphocyte protein; HuCHA60 (HSPD1, HSP60)
Chaperonin-60 complexes with chaperonin-10 & shows weak ATPase activity.
Function:
- implicated in mitochondrial protein import & macromolecular assembly
- may facilitate the correct folding of imported proteins
- may also prevent misfolding & promote the refolding & proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix
- interacts with HBV protein X & HTLV-1 protein p40tax
Structure: belongs to the chaperonin (HSP60) family
Compartment: mitochondrial matrix
Pathology:
- defects in HSPD1 are a cause of spastic paraplegia type-13, autosomal dominant
- defects in HSPD1 are the cause of leukodystrophy hypomyelinating type 4
Laboratory:
- heat shock protein 60 Ab in serum/plasma
General
adenosine triphosphatase (ATPase)
chaperonin; chaperone
heat shock protein
mitochondrial protein
Properties
SIZE: entity length = 573 aa
MW = 61 kD
COMPARTMENT: mitochondrial matrix
MOTIF: Ser phosphorylation site {S70}
Tyr phosphorylation site {Y223}
Tyr phosphorylation site {Y227}
Database Correlations
OMIM correlations
MORBIDMAP 118190
UniProt P10809
Pfam PF00118
Entrez Gene 3329
Kegg hsa:3329
References
- UniProt :accession P10809
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=HSPD1
- Atlas of Genetics & Cytogenetics in Oncology & Haematology
http://atlasgeneticsoncology.org/genes/HSPD1ID40888ch2q33.html