HSPD1 / HSP60抗体

HSPD1 / HSP60抗体产品信息

免疫原 :
Recombinant Mouse HSPD1 / HSP60 protein (Catalog#50596-M07E)
Antibody Type : Rabbit Polyclonal Antibody ( Antibody Purification Platform )
抗体宿主 :
Rabbit IgG
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 :
Produced in rabbits immunized with purified, recombinant Mouse HSPD1 / HSP60 (rM HSPD1 / HSP60; Catalog#50596-M07E; NP_034607.3; Leu2-Phe573). Total IgG was purified by Protein A affinity chromatography.
HSPD1 / HSP60抗体Background
60 kDa heat shock protein, mitochondrial, also known as 60 kDa chaperonin, Chaperonin 60, Heat shock protein 60, HSP-60, Mitochondrial matrix protein P1, HSPD1 and HSP60, is a mitochondrion matrix protein which belongs to thechaperonin (HSP60) family. HSPD1/HSP60 is implicated in mitochondrial protein import and macromolecular assembly. It may facilitate the correct folding of imported proteins. HSPD1/HSP60 may also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix. Defects in HSPD1/HSP60 are a cause of spastic paraplegia autosomal dominant type 13 (SPG13) which is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Defects in HSPD1/HSP60 are also the cause of leukodystrophy hypomyelinating type 4 which 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 two decades of life.
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