ARG59300
anti-Sacsin antibody
anti-Sacsin antibody for Western blot,IHC-Formalin-fixed paraffin-embedded sections and Human,Mouse,Rat
Overview
Product Description | Rabbit Polyclonal antibody recognizes Sacsin |
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Tested Reactivity | Hu, Ms, Rat |
Tested Application | IHC-P, WB |
Host | Rabbit |
Clonality | Polyclonal |
Isotype | IgG |
Target Name | Sacsin |
Antigen Species | Human |
Immunogen | Recombinant protein corresponding to E3709-L3909 of Human Sacsin. |
Conjugation | Un-conjugated |
Alternate Names | DNAJC29; PPP1R138; SPAX6; Sacsin; DnaJ homolog subfamily C member 29; ARSACS |
Application Instructions
Application Suggestion |
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Application Note | IHC-P: Antigen Retrieval: Heat mediated was performed in Citrate buffer (pH 6.0) for 20 min. * The dilutions indicate recommended starting dilutions and the optimal dilutions or concentrations should be determined by the scientist. |
Properties
Form | Liquid |
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Purification | Affinity purification with immunogen. |
Buffer | 0.9% NaCl, 0.2% Na2HPO4, 0.05% Sodium azide and 4% Trehalose. |
Preservative | 0.05% Sodium azide |
Stabilizer | 4% Trehalose |
Concentration | 0.5 - 1 mg/ml |
Storage Instruction | For continuous use, store undiluted antibody at 2-8°C for up to a week. For long-term storage, aliquot and store at -20°C or below. Storage in frost free freezers is not recommended. Avoid repeated freeze/thaw cycles. Suggest spin the vial prior to opening. The antibody solution should be gently mixed before use. |
Note | For laboratory research only, not for drug, diagnostic or other use. |
Bioinformation
Database Links | |
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Gene Symbol | SACS |
Gene Full Name | sacsin molecular chaperone |
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). A pseudogene associated with this gene is located on chromosome 11. Alternative splicing of this gene results in multiple transcript variants. [provided by RefSeq, May 2013] |
Function | Co-chaperone which acts as a regulator of the Hsp70 chaperone machinery and may be involved in the processing of other ataxia-linked proteins. [UniProt] |
Cellular Localization | Cytoplasm. Note=Predominantly cytoplasmic, a small portion is present in the nucleus and also shows a partial mitochondrial overlap with the mitochondrial marker Hsp60. [UniProt] |
Calculated MW | 521 kDa |