ITN2497
ITN2497
ITN2497
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- Catalog: ITN2497
- Gene/Protein: SACS KIAA0730
- Product Description: Immunotag™ SACS Polyclonal Antibody
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Description
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Specifications
Antibody Specification Datasheet 
IMPORTANT NOTE This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. Target Protein SACS Clonality Polyclonal Storage/Stability -20°C/1 year Application IHC-p Recommended Dilution IHC-p 1:50-300 Concentration 1 mg/ml Reactive Species Human,Mouse Host Species Rabbit Immunogen Synthesized peptide derived from human protein . at AA range: 4291-4340 Specificity SACS Polyclonal Antibody detects endogenous levels of protein. Purification The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen Form Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. Gene Name SACS KIAA0730 Accession No. Q9NZJ4 Q9JLC8 Description sacsin molecular chaperone(SACS) Homo sapiens 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). Protein Expression Astrocyte,Brain,Fetal liver,Uterine endothelium, Subcellular Localization nucleus,cytoplasm,mitochondrion,axon,dendrite,cell body fiber, Protein 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., Usage For Research Use Only! Not for diagnostic or therapeutic procedures. - Reviews
| Antibody Specification | |
| Datasheet | |
| IMPORTANT NOTE | This product is custom manufactured with a lead time of 3-4 weeks. Once in production, this item cannot be cancelled from an order and is not eligible for return. |
| Target Protein | SACS |
| Clonality | Polyclonal |
| Storage/Stability | -20°C/1 year |
| Application | IHC-p |
| Recommended Dilution | IHC-p 1:50-300 |
| Concentration | 1 mg/ml |
| Reactive Species | Human,Mouse |
| Host Species | Rabbit |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 4291-4340 |
| Specificity | SACS Polyclonal Antibody detects endogenous levels of protein. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen |
| Form | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Gene Name | SACS KIAA0730 |
| Accession No. | Q9NZJ4 Q9JLC8 |
| Description | sacsin molecular chaperone(SACS) Homo sapiens 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). |
| Protein Expression | Astrocyte,Brain,Fetal liver,Uterine endothelium, |
| Subcellular Localization | nucleus,cytoplasm,mitochondrion,axon,dendrite,cell body fiber, |
| Protein 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., |
| Usage | For Research Use Only! Not for diagnostic or therapeutic procedures. |