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.
Formulation
0.5mg/ml if reconstituted with 0.2ml sterile DI water
Host
Rabbit
Immunogen Region
A recombinant human partial protein corresponding to amino acids E3709-L3909 was used as the immunogen for the Sacsin antibody.
Isotype
IgG
Predicted Reactivity
Human, Mouse, Rat
Reactivity
Human, Mouse, Rat
Recombinant
No
Subcellular Location
Cytoplasmic
Uniprot
Q9NZJ4
Buffer
Lyophilized from 1X PBS with 2% Trehalose and 0.025% sodium azide
Format
Antigen affinity purified
Purification
Antigen affinity purified
Storage
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.
Applications
WB, IHC-P, ELISA
Dilution
Western Blot: 0.5-1ug/ml,Immunohistochemistry (FFPE): 1-2ug/ml,Direct ELISA: 0.1-0.5ug/ml