Recombinant人Hsp60 protein (ab113192)

常规信息

• 别名
  • 60 kDa chaperonin
  • 60 kDa heat shock protein, mitochondrial
  • CH60_HUMAN

  • Chaperonin 60
  • Chaperonin, 60-KD
  • CPN60
  • fa04a05
  • GROEL
  • heat shock 60kDa 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

  see all
• 功能
  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.
• 疾病相关
  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.
  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 two decades of life.
• 序列相似性
  Belongs to the chaperonin (HSP60) family.
• 细胞定位
  Mitochondrion matrix.

• Target information above from: UniProt accession P10809 The UniProt Consortium
  The Universal Protein Resource (UniProt) in 2010
  Nucleic Acids Res. 38:D142-D148 (2010) .

  Information by UniProt