Scientific Background: HSP60 is a chaperonin protein, required for ATP-dependent folding of precursor polypeptides and complex assembly and prevents aggregation and mediates protein refolding after heat shock. Mutations and variations in the HSP 60 gene product may be implicated in genetic diseases (1). HSP60 has been strongly implicated as an example of molecular mimicry in the pathogenicity of autoimmune diseases in T cell-mediated protection. Human HSP60 was found to activate the complement system in normal human serum in a dose-dependent manner. Since complement activation plays an important role in the development of atherosclerosis, the levels of complement-activating anti-HSP60 antibodies are elevated in atherosclerosis-related diseases. (2)
2. Zolta¡n, P. et al: Antibodies against human heat-shock protein (hsp) 60 and mycobacterial hsp65 differ in their antigen specificity and complement-activating abililty. Int. Immunol. 1999;11 (9), 1363-1370.
NCBI and Uniprot Product Information
NCBI Description
This gene encodes a member of the chaperonin family. The encoded mitochondrial protein may function as a signaling molecule in the innate immune system. This protein is essential for the folding and assembly of newly imported proteins in the mitochondria. This gene is adjacent to a related family member and the region between the 2 genes functions as a bidirectional promoter. Several pseudogenes have been associated with this gene. Two transcript variants encoding the same protein have been identified for this gene. Mutations associated with this gene cause autosomal recessive spastic paraplegia 13. [provided by RefSeq, Jun 2010]
Uniprot Description
Function: Implicated in mitochondrial protein import and macromolecular assembly. May facilitate the correct folding of imported proteins. May also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix.
Subunit structure: Interacts with HRAS
By similarity. Interacts with HBV protein X and HTLV-1 protein p40tax. Interacts with ATAD3A. Interacts with METTL20 and METTL21B. Ref.9 Ref.19 Ref.29 Ref.30
Subcellular location: Mitochondrion matrix.
Involvement in disease: Spastic paraplegia 13, autosomal dominant (SPG13) [MIM:605280]: A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.31Leukodystrophy, hypomyelinating, 4 (HLD4) [MIM:612233]: A severe autosomal recessive hypomyelinating leukodystrophy. Clinically characterized by infantile-onset rotary nystagmus, progressive spastic paraplegia, neurologic regression, motor impairment, profound mental retardation. Death usually occurs within the first two decades of life.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.32
Sequence similarities: Belongs to the chaperonin (HSP60) family.
Research Articles on HSP60
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Product Notes
The HSP60 hspd1 (Catalog #AAA515419) is a Recombinant Protein produced from E Coli and is intended for research purposes only. The product is available for immediate purchase. The tag for this protein is GST tag!!Expression System||E.coli!!Source Note||Recombinant full-length human HSP60 was expressed in E. coli cells. AAA Biotech's HSP60 can be used in a range of immunoassay formats including, but not limited to, Kinase Assay, Western Blot (WB). Researchers should empirically determine the suitability of the HSP60 hspd1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "HSP60, Recombinant Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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