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
限制
仅限研究用
缓冲液
0.5 mg/mL if reconstituted with 0.2 mL sterile DI water
储存条件
-20 °C
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.
抗原
Sacsin (SACS)
(Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))
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.