Sacsin 抗体
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Quick Overview for Sacsin 抗体 (ABIN5708501)
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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纯化方法
- Antigen affinity purified
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免疫原
- A recombinant human partial protein corresponding to amino acids E3709-L3909 was used as the immunogen for the Sacsin antibody.
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亚型
- IgG
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应用备注
- Optimal dilution of the Sacsin antibody should be determined by the researcher.\. Western Blot: 0.5-1 μg/mL,IHC (FFPE): 1-2 μg/mL,Direct ELISA: 0.1-0.5 μg/mL
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限制
- 仅限研究用
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缓冲液
- 0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
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储存条件
- -20 °C
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储存方法
- After reconstitution, the Sacsin antibody can be stored for up to one month at 4°C. For long-term, aliquot and store at -20°C. Avoid repeated freezing and thawing.
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- Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))
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别名
- Sacsin
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背景
- Sacsin also known as DnaJ homolog subfamily C member 29 (DNAJC29) is a protein that in humans is encoded by the SACS gene. This gene consists of nine exons including a gigantic exon spanning more than 12.8k bp. It encodes the sacsin protein, which includes a UBQ region at the N-terminus, a HEPN domain at the C-terminus and a DnaJ region upstream of the HEPN domain. This modular protein is essential for normal mitochondrial network organization. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. Mutations in this gene result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticityand peripheral neuropathy.
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UniProt
- Q9NZJ4
抗原
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