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-terminal, 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.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 0.1-0.5ug/mlWestern Blot: 0.1-0.5ug/mlImmunohistochemistry (paraffin): 0.5-1ug/mlOptimal dilutions to be determined by the researcher.
Storage and Stability:Lyophilized powder may be stored at -20°C.
Stable for 12 months after receipt at -20°C.
Reconstitute with sterile ddH2O.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Reconstituted product is stable for 12 months at -20°C.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Further dilutions can be made in assay buffer.
仕様
Size:100ug
Host:rabbit
Source Antibody:human
Grade:Affinity Purified
Purity:Purified by immunoaffinity chromatography.
Form:Supplied as a lyophilized powder in 4mg trehalose, 0.9mg sodium chloride, 0.2mg Na2HPO4, 0.05% sodium azide. Reconstitute with 200ul sterile ddH2O to ~0.5mg/ml.
Specificity:Recognizes human SACS. Species Crossreactivity: mouse, rat.
Isotype:IgG
Calc Applications Abbrev:E IHC WB
Calc Crossreactivity:Hu Mo Rt
Immunogen:Recombinant protein corresponding to human SACS, (Position: E3709-L3909), expressed in E. coli.