CABC1 Polyclonal Antibody
- 货号：YT0583
- 应用：WB;IHC;IF;ELISA
- 种属：Human;Mouse;Rat
- 蛋白名称：
- Chaperone activity of bc1 complex-like mitochondrial
- 免疫原：
- The antiserum was produced against synthesized peptide derived from human ADCK3. AA range:301-350
- 特异性：
- CABC1 Polyclonal Antibody detects endogenous levels of CABC1 protein.
- 组成：
- Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
- 来源：
- Polyclonal, Rabbit,IgG
- 稀释：
- WB 1:500 - 1:2000. IHC 1:100 - 1:300. IF 1:200 - 1:1000. ELISA: 1:10000. Not yet tested in other applications.
- 纯化工艺：
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
- 储存：
- -15°C to -25°C/1 year(Do not lower than -25°C)
- 其他名称：
- ADCK3;CABC1;PP265;Chaperone activity of bc1 complex-like; mitochondrial;Chaperone-ABC1-like;aarF domain-containing protein kinase 3
- 背景：
- 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],
- 功能：
- 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. Patie
- 细胞定位：
- Mitochondrion . Membrane ; Single-pass membrane protein .
- 组织表达：
- Widely expressed, with highest levels in adrenal gland, heart, pancreas, nasal mucosa, stomach, uterus and skeletal muscle.
- Western Blot analysis of various cells using CABC1 Polyclonal Antibody
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- Western Blot analysis of COLO205 cells using CABC1 Polyclonal Antibody
- Western blot analysis of lysates from HepG2 and mouse muscle cells, using ADCK3 Antibody. The lane on the right is blocked with the synthesized peptide.
- Immunohistochemical analysis of paraffin-embedded human tonsil. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 30min).
