概述

描述

  • 性质
    Recombinant
  • 来源
    Escherichia coli
  • 氨基酸序列
    • Accession
      P10809
    • 种属
      Human
    • 分子量
      60 kDa
    • 氨基酸
      1 to 573

技术指标

Our Abpromise guarantee covers the use of ab113192 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

  • 生物活性
    ATPase activity assay (positive)but there is not quantification of activity
  • 应用

    Western blot

    Peptide Array

    SDS-PAGE

    Functional Studies

    ELISA

  • 内毒素水平
    > 500.000 Eu/mg
  • 纯度
    > 90 % SDS-PAGE.
    ab113192 is purified by multi-step chromatography.
  • 形式
    Liquid
  • Concentration information loading...

制备和贮存

  • 稳定性和存储

    Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.

    Constituent: 100% PBS
    Note: Dulbecco's PBS.

    This product is an active protein and may elicit a biological response in vivo, handle with caution.

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.
  • Information by UniProt

图片

  • SDS-PAGE - Hsp60 protein (ab113192)
    SDS-PAGE - Hsp60 protein (ab113192)
    SDS-PAGE showing ab113192 at approximately 60kDa (1µg).
  • Western blot - Hsp60 protein (ab113192)
    Western blot - Hsp60 protein (ab113192)
    All lanes : anti-Hsp60 mAb at 1 µg/ml

    Lane 1 : Recombinant human Hsp60 protein (ab113192)
    Lane 2 : GroEL protein

    Lysates/proteins at 0.1 µg per lane.

文献

ab113192 has not yet been referenced specifically in any publications.

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