{"product_id":"es11443","title":"SACS rabbit pAb","description":"\u003cstrong\u003eApplications:\u003c\/strong\u003e IHC;IF\u003cbr\u003e\u003cstrong\u003eReactivity:\u003c\/strong\u003e Human;Mouse\u003cbr\u003e\u003cstrong\u003eSource:\u003c\/strong\u003e Rabbit\u003cbr\u003e\u003cstrong\u003eDilution:\u003c\/strong\u003e IHC-p 1:50-300\u003cbr\u003e\u003cstrong\u003eImmunogen:\u003c\/strong\u003e Synthesized peptide derived from human protein . at AA range: 4291-4340\u003cbr\u003e\u003cstrong\u003eStorage_stability:\u003c\/strong\u003e -20°C\/1 year\u003cbr\u003e\u003cstrong\u003eClonality:\u003c\/strong\u003e Polyclonal\u003cbr\u003e\u003cstrong\u003eIsotype:\u003c\/strong\u003e IgG\u003cbr\u003e\u003cstrong\u003eConcentration:\u003c\/strong\u003e 1 mg\/ml\u003cbr\u003e\u003cstrong\u003eObserved_band(KD):\u003c\/strong\u003e 503kD\u003cbr\u003e\u003cstrong\u003eHuman_gene_id:\u003c\/strong\u003e 26278\u003cbr\u003e\u003cstrong\u003eHuman_swiss_prot_no:\u003c\/strong\u003e Q9NZJ4\u003cbr\u003e\u003cstrong\u003eSubcellular_location:\u003c\/strong\u003e Cytoplasm . Predominantly cytoplasmic, a small portion is present in the nucleus and also shows a partial mitochondrial overlap with the mitochondrial marker Hsp60.\u003cbr\u003e\u003cstrong\u003eBackground:\u003c\/strong\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).","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":50413169770776,"sku":"ES11443","price":250.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/ES11443-c-1.jpg?v=1751141525","url":"https:\/\/danabiosci.com\/products\/es11443","provider":"Dana Bioscience","version":"1.0","type":"link"}