参阅全部 Hsp60 蛋白酶
蛋白长度Full length protein
氨基酸1 to 573
Our Abpromise guarantee covers the use of ab113177 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
内毒素水平< 50.000 Eu/mg
纯度> 90 % SDS-PAGE.
ab113177 was purified by multi-step chromatography.
Concentration information loading...
Shipped on dry ice. Upon delivery aliquot and store at -80ºC. Avoid freeze / thaw cycles.
Preservative: 0.09% Sodium azide
Constituents: 99% PBS, Phosphate Buffer
- 60 kDa chaperonin
- 60 kDa heat shock protein, mitochondrial
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.
- Information by UniProt
SDS-PAGE analysis of ab113177: Lane 1: MW marker, Lane 2: 0.5ug, Lane 3: 1ug, Lane 4: 2.5ug, Lane 5: 5ug, Lane 6: 10ug.
All lanes : a monoclonal anti Hsp60 protein
Lane 1 : molecualr weight marker
Lane 2 :
Recombinant Human Hsp60 protein (ab113177) at 0.1 µg
Lane 3 :
Recombinant Human Hsp60 protein (ab113177) at 0.05 µg
ab113177 has not yet been referenced specifically in any publications.