Gene Name：HSPD1 HSP60
Protein Name：60 kDa heat shock protein, mitochondrial (60 kDa chaperonin) (Chaperonin 60) (CPN60) (Heat shock protein 60) (HSP-60) (Hsp60) (HuCHA60) (Mitochondrial matrix protein P1) (P60 lymphocyte protein)
Human Gene Id：3329
Human Swiss Prot No：P10809
Immunogen：Synthesized peptide derived from human HSP60
Specificity：This antibody detects endogenous levels of human HSP60
Formulation：Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
Purification：The antibody was affinity-purified from mouse ascites by affinity-chromatography using specific immunogen.
Storage Stability：-20°C/1 year
Background: heat shock protein family D (Hsp60) member 1(HSPD1) Homo sapiens 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],
Function: disease: defects in HSPD1 are a cause of spastic paraplegia autosomal dominant type 13 (SPG13) [MIM:605280]. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs, disease: defects in HSPD1 are the cause of leukodystrophy hypomyelinating type 4 (HLD4) [MIM:612233]; also called mitochondrial HSP60 chaperonopathy or MitCHAP-60 disease. HLD4 is 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 occurrs within the first 2 decades of life, function: Implicated in mitochondrial protein import and macromolecular assembly. May facilitate the correct folding of imported proteins. May also prevent misfolding and promote.
Subcellular Location: Cytoplasmic.
Expression: Adipocyte, Adrenal gland, B-cell lymphoma, Brain, Cajal-Retzius.
1. Store at -20 ℃ for one year. Avoid repeated freeze/thaw cycles.
2. This product is intended for research only.