BD-PT0583

CABC1 Rabbit Polyclonal Antibody

ADCK3; CABC1; PP265; Chaperone activity of bc1 complex-like; mitochondrial; Chaperone-ABC1-like; aarF domain-containing protein kinase 3

常规抗体

ADCK3

Chaperone activity of bc1 complex-like mitochondrial

WB

Human,Mouse,Rat

1 mg/ml

Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% New type preservative N.

56997

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=56997

Q8NI60

http://www.uniprot.org/uniprotkb/Q8NI60/entry

67426

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=67426

Q60936

http://www.uniprot.org/uniprot/Q60936

360887

http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&term=360887

Q5BJQ0

http://www.uniprot.org/uniprot/Q5BJQ0

The antiserum was produced against synthesized peptide derived from human ADCK3. AA range:301-350

CABC1 Polyclonal Antibody detects endogenous levels of CABC1 protein.

WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.

70kD

-20°C/1 year

Polyclonal, Rabbit,IgG

This gene encodes a mitochondrial protein similar to yeast ABC1, which functions in an electron-transferring membrane protein complex in the respiratory chain. It is not related to the family of ABC transporter proteins. Expression of this gene is induced by the tumor suppressor p53 and in response to DNA damage, and inhibiting its expression partially suppresses p53-induced apoptosis. Alternatively spliced transcript variants have been found; however, their full-length nature has not been determined. [provided by RefSeq, Jul 2008],

Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.

Mitochondrion . Membrane ; Single-pass membrane protein .

disease:Defects in CABC1 are a cause of coenzyme Q10 deficiency [MIM:607426]; also known as primary CoQ10 deficiency. Coenzyme Q10 deficiency patients present a progressive neurological disorder with cerebellar atrophy, developmental delay, and hyperlactatemia.,disease:Defects in CABC1 are the cause of spinocerebellar ataxia autosomal recessive type 9 (SCAR9) [MIM:612016]; also known as autosomal recessive cerebellar ataxia type 2 (ARCA2). Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCAR9 is an autosomal recessive form characterized by gait ataxia and cerebellar atrophy with slow progression and few associated features. Patients can manifest brisk tendon reflexes and Hoffmann sign, mild psychomotor retardation, mild axonal degeneration of the sural nerve, exercise intolerance and elevated serum lactate.,function:May be a chaperone-like protein essential for the proper conformation and functioning of protein complexes in the respiratory chain.,induction:By p53.,similarity:Belongs to the protein kinase superfamily. ADCK protein kinase family.,similarity:Contains 1 protein kinase domain.,tissue specificity:Ubiquitously expressed with a relatively greater abundance in heart and skeletal muscle.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.