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HSPD1 / HSP60 Antibody, Rabbit PAb, Antigen Affinity Purified

  • HSPD1 / HSP60 Antibody, Rabbit PAb, Antigen Affinity Purified, Western blot
  • HSPD1 / HSP60 Antibody, Rabbit PAb, Antigen Affinity Purified, Immunofluorescence
  • HSPD1 / HSP60 Antibody, Rabbit PAb, Antigen Affinity Purified, Immunoprecipitation
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Human HSPD1 Antibody Product Information
Immunogen:A synthetic peptide corresponding to the C-terminus of the Human HSPD1 / HSP60
Clone ID:
Ig Type:Rabbit IgG
Concentration:
Endotoxin:
Formulation:0.2 μm filtered solution in PBS
Preparation:Produced in rabbits immunized with a synthetic peptide corresponding to the C-terminus of the Human HSPD1 / HSP60, and purified by antigen affinity chromatography.
Human HSPD1 Antibody WB Application Image
Human HSPD1 Antibody IF Application Image
HSPD1 / HSP60 Antibody, Rabbit PAb, Antigen Affinity Purified, Immunofluorescence
[Click to enlarge image]
Caption:
Immunofluorescence staining of HSPD1 in HeLa cells. Cells were fixed with 4% PFA,blocked with 10% serum, and incubated with rabbit anti-human HSPD1 polyclonal antibody (1 µg/ml) at 4℃ overnight. Then cells were stained with the Alexa Fluor®488-conjugated
Human HSPD1 Antibody IP Application Image
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HSPD1/HSP60 Background

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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Human HSPD1/HSP60 References
  • Hansen J J, et al. (2002) Hereditary spastic paraplegia SPG13 is associated with a mutation in the gene encoding the mitochondrial chaperonin Hsp60. Am J Hum Genet. 70: 1328-32.
  • Magen D, et al. (2008) Mitochondrial Hsp60 chaperonopathy causes an autosomal-recessive neurodegenerative disorder linked to brain hypomyelination and leukodystrophy. Am J Hum Genet. 83: 30-42.
  • Venner TJ, et al. (1990) Nucleotide sequences and novel structural features of human and Chinese hamster hsp60 (chaperonin) gene families. DNA Cell Biol. 9 (8): 545-52.
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    Catalog: 101240-T38-100
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    Datasheet & Documentation

    All information of our products is subject to change without notice. Please refer to COA enclosed in shipped package for the newest information.
    Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"