Sacsin 抗体 (AA 4291-4340)
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Quick Overview for Sacsin 抗体 (AA 4291-4340) (ABIN7231244)
抗原
See all Sacsin (SACS) 抗体适用
宿主
克隆类型
标记
应用范围
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抗原表位
- AA 4291-4340
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原理
- Rabbit Anti-SACS Polyclonal Antibody
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特异性
- The antibody detects endogenous levels of SACS protein
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纯化方法
- The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
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免疫原
- Synthesized peptide derived from part region of human SACS protein at AA range: 4291-4340
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亚型
- IgG
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应用备注
- Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: IHC 1:50-300,IF 1:50-200
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限制
- 仅限研究用
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状态
- Liquid
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浓度
- 1 mg/mL
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缓冲液
- PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 %BSA
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储存液
- ProClin
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注意事项
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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储存条件
- -20 °C
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储存方法
- Stable for one year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Aliquot to avoid repeated freezing and thawing.
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有效期
- 12 months
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- Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))
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别名
- SACS
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背景
- Sacsin, DnaJ homolog subfamily C member 29, DNAJC29SACS encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. SACS is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. SACS includes a very large exon spanning more than 12. kb. Mutations in SACS result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins" (Parfitt et al. PubMed: 19208651). A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants.
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分子量
- 503kD
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基因ID
- 26278
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UniProt
- Q9NZJ4
抗原
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