• SACS Polyclonal Antibody
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YP-Ab-07351-100UL

SACS Polyclonal Antibody

SACS Polyclonal Antibody

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  • Gene Name: SACS KIAA0730
  • Immunogen: Synthesized peptide derived from human protein . at AA range: 4291-4340
  • Specificity: SACS Polyclonal Antibody detects endogenous levels of protein.
  • Composition: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
  • Source: Polyclonal, Rabbit,IgG
  • Dilution ratio: IHC-p 1:50-300. IF 1:50-200
  • Purification process: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
  • Concentration: 1 mg/ml
  • Storage: -20°C/1 year
  • Background: This gene encodes the sacsin protein, which includes a UbL domain at the N-terminus, a DnaJ domain, and a HEPN domain at the C-terminus. The gene is highly expressed in the central nervous system, also found in skin, skeletal muscles and at low levels in the pancreas. This gene includes a very large exon spanning more than 12.8 kb. Mutations in this gene 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).
  • Function: disease:Defects in SACS are the cause of autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) [MIM:270550]. ARSACS is an early onset neurodegenerative disease with high prevalence in the Charlevoix-Saguenay-Lac-Saint-Jean region of Quebec. It is characterized by absent sensory-nerve conduction, reduced motor-nerve velocity and hypermyelination of retinal-nerve fibers.,function:May function in chaperone-mediated protein folding.,similarity:Contains 1 HEPN domain.,similarity:Contains 1 J domain.,tissue specificity:Highly expressed in the central nervous system. Also found in skeletal muscle and at low levels in pancreas.,
  • Species: Human;Mouse
  • Range: IHC;IF
  • Protein: Sacsin (DnaJ homolog subfamily C member 29) (DNAJC29)
  • DA: 503kD
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