{"product_id":"sacs-rabbit-pab-antibody","title":"SACS rabbit pAb","description":"\u003cp\u003e\u003cb\u003eSize\u003c\/b\u003e: 100μL\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSource\u003c\/b\u003e:Rabbit\u003c\/p\u003e\u003cp\u003e\u003cb\u003eApplications\u003c\/b\u003e:IHC;IF\u003c\/p\u003e\u003cp\u003e\u003cb\u003eReactivity\u003c\/b\u003e:Human;Mouse\u003c\/p\u003e\u003cp\u003e\u003cb\u003eDilution\u003c\/b\u003e:IHC-p 1:50-300\u003c\/p\u003e\u003cp\u003e\u003cb\u003eImmunogen\u003c\/b\u003e:Synthesized peptide derived from human protein . at AA range: 4291-4340\u003c\/p\u003e\u003cp\u003e\u003cb\u003eStorage_stability\u003c\/b\u003e:-20°C\/1 year\u003c\/p\u003e\u003cp\u003e\u003cb\u003eClonality\u003c\/b\u003e:Polyclonal\u003c\/p\u003e\u003cp\u003e\u003cb\u003eIsotype\u003c\/b\u003e:IgG\u003c\/p\u003e\u003cp\u003e\u003cb\u003eConcentration\u003c\/b\u003e:1 mg\/ml\u003c\/p\u003e\u003cp\u003e\u003cb\u003eObserved_band(KD)\u003c\/b\u003e:503kD\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHuman_gene_id\u003c\/b\u003e:26278\u003c\/p\u003e\u003cp\u003e\u003cb\u003eHuman_swiss_prot_no\u003c\/b\u003e:Q9NZJ4\u003c\/p\u003e\u003cp\u003e\u003cb\u003eSubcellular_location\u003c\/b\u003e:Cytoplasm . Predominantly cytoplasmic, a small portion is present in the nucleus and also shows a partial mitochondrial overlap with the mitochondrial marker Hsp60.\u003c\/p\u003e\u003cp\u003e\u003cb\u003eOther_name\u003c\/b\u003e:\u003c\/p\u003e\u003cp\u003e\u003cb\u003eBackground\u003c\/b\u003e: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).\u003c\/p\u003e","brand":"GeneBio Systems","offers":[{"title":"Default Title","offer_id":48647432863844,"sku":"ES11443","price":325.95,"currency_code":"EUR","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0558\/8588\/9636\/files\/no_image_default_image-jpeg_e21c399d-9e4c-483b-a910-3ea2b3d394b5.jpg?v=1782698803","url":"https:\/\/www.genebiosystems.com\/en-fr\/products\/sacs-rabbit-pab-antibody","provider":"GeneBio ","version":"1.0","type":"link"}