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    2. 重組蛋白產品

      名稱 Human HSPD1 / HSP60 Protein/重組人 HSPD1 / HSP60
      規格 20ug
      編號 CZDB-012
      價格 詢價
      中文名 Human HSPD1 / HSP60 Protein/重組人 HSPD1 / HSP60
      分子別稱 HSPD1, CPN60, GROEL, HLD4, HSP60, HSP65, HuCHA60,
      分子種屬 Mouse
      表達標簽 His,S
      表達宿主 E. coli
      濃度 > 95 % as determined by SDS-PAGE
      緩沖液 Lyophilized from sterile PBS, pH 7.4
      存儲條件 -70°C,應避免反復凍融。
      基本描述 HSPD1, also known as HSP60, is a member of the chaperonin family. HSPD1 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. It may also prevent misfolding and promote the refolding and proper assembly of unfolded polypeptides generated under stress conditions in the mitochondrial matrix. HSPD1 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. Mutations associated with this gene cause autosomal recessive spastic paraplegia 13.Defects in HSPD1 are a cause of spastic paraplegia autosomal dominant type 13 (SPG13). 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); also called mitochondrial HSP60 chaperonopathy or MitCHAP-60 disease. HLD4 is a severe autosomal recessive hypomyelinating leukodystrophy. HSPD1 is cinically 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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