Sacsin antibody (AA 4291-4340)

Catalog No. ABIN7231244

The Rabbit Polyclonal anti-Sacsin antibody is suitable to detect Sacsin in samples from Human and Mouse. It has been validated for IHC and IF.

Quick Overview for Sacsin antibody (AA 4291-4340) (ABIN7231244)

Target: Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))

Reactivity: Human, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This Sacsin antibody is un-conjugated

Application: Immunohistochemistry (IHC), Immunofluorescence (IF)

Product Details anti-Sacsin Antibody

Binding Specificity: AA 4291-4340

Purpose: Rabbit Anti-SACS Polyclonal Antibody

Specificity: The antibody detects endogenous levels of SACS protein

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Immunogen: Synthesized peptide derived from part region of human SACS protein at AA range: 4291-4340

Isotype: IgG

Application Details

Application Notes: Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: IHC 1:50-300,IF 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 mg/mL

Buffer: PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 %BSA

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Stable for one year at -20°C from date of shipment. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Aliquot to avoid repeated freezing and thawing.

Expiry Date: 12 months

Target Details for Sacsin

Target: Sacsin (SACS) (Spastic Ataxia of Charlevoix-Saguenay (Sacsin) (SACS))

Alternative Name: SACS

Background: Sacsin, DnaJ homolog subfamily C member 29, DNAJC29SACS encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. SACS is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. SACS includes a very large exon spanning more than 12. kb. Mutations in SACS result in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS), a neurodegenerative disorder characterized by early-onset cerebellar ataxia with spasticity and peripheral neuropathy. The authors of a publication on the effects of siRNA-mediated sacsin knockdown concluded that sacsin protects against mutant ataxin-1 and suggest that "the large multi-domain sacsin protein is able to recruit Hsp70 chaperone action and has the potential to regulate the effects of other ataxia proteins" (Parfitt et al. PubMed: 19208651). A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants.

Molecular Weight: 503kD

Gene ID: 26278

UniProt: Q9NZJ4