HSPD1 / HSP60 Protein & Antibody

Heat Shock 60kDa Protein 1 (chaperonin)

HSPD1 / HSP60 Products

HSPD1 / HSP60 Protein, Recombinant

Molecule	Species	Description	Cat. No
HSPD1/HSP60	Human	HSPD1/HSP60 Protein, Recombinant	11322-H07E
HSPD1/HSP60	Mouse	HSPD1/HSP60 Protein, Recombinant	50596-M07E

HSPD1 / HSP60 Antibody

Molecule	Application	Description	Cat. No
Human HSPD1/HSP60	WB, ELISA, IHC-P	HSPD1 / HSP60 Antibody (Antigen Affinity Purified)	50596-RP02

HSPD1 / HSP60 cDNA Clone

Molecule	Species	Description	Cat. No
HSPD1/HSP60	Human	Homo sapiens HSPD1/HSP60 cDNA Clone	HG11322-M
HSPD1/HSP60	Mouse	HSPD1 cDNA Clone / ORF Clone	MG50596-M

HSPD1 / HSP60 Related Areas

Cancer>>Apoptosis>>Heat-Shock Protein>>HSPD1/HSP60

HSPD1 / HSP60 Alternative Names

HSPD1, HSP60, CPN60, GROEL, HLD4, HSP65, HuCHA60, SPG13 [Homo sapiens]

Hspd1, Hsp60, 60kDa [Mus musculus]

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.

HSPD1 / HSP60 Related Studies

1. 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.
2. 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.
3. 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.