免疫原
Purified recombinant human Hsp60 protein fragments expressed in E.coli.
特异性
This antibody detects endogenous levels of Hsp60 and does not cross-react with related proteins.
组成
Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
稀释比例
wb dilution 1:1000 icc dilution 1:100 ip dilution 1:100. IF 1:50-200
纯化工艺
The antibody was affinity-purified from mouse ascites by affinity-chromatography using epitope-specific immunogen.
其他名称
60 kDa chaperonin;60 kDa heat shock protein mitochondrial;60 kDa heat shock protein, mitochondrial;CH60_HUMAN;Chaperonin 60;Chaperonin;Chaperonin, 60-KD;CPN 60;CPN60;fa04a05;GROEL;GroEL Homolog;Heat shock 60kD protein 1 chaperonin;Heat shock 60kDa protein 1;Heat shock protein 1 (chaperonin);Heat Shock Protein 60;Heat shock protein 65;HLD4;Hsp 60;HSP 65;HSP-60;HSP60;HSP65;HSPD 1;HSPD1;HuCHA60;Mitochondrial matrix protein P1;P60 lymphocyte protein;Short heat shock protein 60 Hsp60s1;Spastic paraplegia 13;SPG 13;SPG13.
背景
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],
功能
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 the
