The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
ATPase activity assay (positive)but there is not quantification of activity
> 500.000 Eu/mg
% SDS-PAGE. ab113192 is purified by multi-step chromatography.
Concentration information loading...
Preparation and Storage
Stability and Storage
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.
60 kDa chaperonin
60 kDa heat shock protein, mitochondrial
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
Mitochondrial matrix protein P1
P60 lymphocyte protein
short heat shock protein 60 Hsp60s1
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.
Involvement in disease
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.