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397542

SACS (Sacsin, DnaJ Homolog Subfamily C Member 29, DNAJC29, KIAA0730)

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£620.00

SKU:
397542
Reinheit:
Purified by immunoaffinity chromatography.
Lagerbedingungen:
-20°C
Hersteller:
United States Biological
Host:
Rabbit
Immunogen:
Recombinant protein corresponding to human SACS, (Position: E3709-L3909), expressed in E. coli.
Format:
Supplied as a lyophilized powder from PBS, 0.05% sodium azide, 4% trehalose. Reconstitute with 200ul sterile ddH2O.
Weitere Details:
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/ml Western Blot: 0.1-0.5ug/ml Immunohistochemistry (paraffin): 0.5-1ug/ml Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized and reconstituted products are stable for 12 months after receipt at -20°C. Reconstitute with sterile ddH2O. Aliquot to avoid repeated freezing and thawing. Store 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.
Versandbedingungen:
Blue Ice