蛋白名称
60 kDa heat shock protein mitochondrial
特异性
This antibody detects endogenous levels of Hsp60
组成
The prediluted ready-to-use antibody is diluted in phosphate buffer saline containing stabilizing protein and 0.05% Proclin 300
来源
Monoclonal, Rabbit,IgG
稀释比例
Ready to use for IHC Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0
纯化工艺
Recombinant Expression and Affinity purified
储存
2°C to 8°C/1 year,Ship by ice bag
其他名称
60 kDa chaperonin ; 60 kDa heat shock protein, mitochondrial ; CH60_HUMAN ; Chaperonin 60 ; Chaperonin, 60-KD ; CPN60 ; fa04a05 ; GROEL ; heat shock 60 kDa protein 1 ; chaperonin ; Heat shock protein 1 ; chaperonin ; Heat shock protein 60 ; Heat shock protein 65 ; heat shock protein family D ; Hsp60 ; member 1 ; HLD4 ; Hsp 60 ; HSP 65 ; HSP-60 ; HSP60 ; HSP65 ; HSPD1 ; HuCHA60 ; Mitochondrial matrix protein P1 ; P60 lymphocyte protein ; short heat shock protein 60 Hsp60s1 ; 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 refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix.,similarity:Belongs to the chaperonin (HSP60) family.,similarity:Belongs to the TCP-1 chaperonin family.,subunit:Interacts with HBV protein X and HTLV-1 protein p40tax.,
