{"product_id":"yp-ab-05911-100ul","title":"PEX6 Polyclonal Antibody","description":"\u003cul\u003e\n\u003cli\u003e\n\u003cb\u003eGene Name:\u003c\/b\u003e PEX6 PXAAA1\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eImmunogen:\u003c\/b\u003e Synthesized peptide derived from human protein . at AA range: 480-560\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecificity:\u003c\/b\u003e PEX6 Polyclonal Antibody detects endogenous levels of protein.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eComposition:\u003c\/b\u003e Liquid in PBS containing 50% glycerol,  and 0.02% sodium azide.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSource:\u003c\/b\u003e Polyclonal, Rabbit,IgG\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDilution ratio:\u003c\/b\u003e WB 1:500-2000 ELISA 1:5000-20000\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003ePurification process:\u003c\/b\u003e The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eConcentration:\u003c\/b\u003e 1 mg\/ml\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eStorage:\u003c\/b\u003e -20°C\/1 year\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eBackground:\u003c\/b\u003e This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Oct 2015],\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eFunction:\u003c\/b\u003e disease:Defects in PEX6 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life.,disease:Defects in PEX6 are the cause of peroxisome biogenesis disorder complementation group 4 (PBD-CG4) [MIM:601498]; also known as PBD-CGC. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical cont\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eSpecies:\u003c\/b\u003e Human;Rat;Mouse\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eRange:\u003c\/b\u003e WB;ELISA\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eProtein:\u003c\/b\u003e Peroxisome assembly factor 2 (PAF-2) (Peroxin-6) (Peroxisomal biogenesis factor 6) (Peroxisomal-type ATPase 1)\u003c\/li\u003e\n\u003cli\u003e\n\u003cb\u003eDA:\u003c\/b\u003e 107kD\u003c\/li\u003e\n\u003c\/ul\u003e","brand":"Upingbio","offers":[{"title":"100UL","offer_id":50690088927512,"sku":"YP-Ab-05911-100UL","price":0.0,"currency_code":"USD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0590\/5652\/1400\/files\/images_741f73cd-4388-4e61-a50b-4a1ef0b6b4be.png?v=1758597814","url":"https:\/\/danabiosci.com\/products\/yp-ab-05911-100ul","provider":"Dana Bioscience","version":"1.0","type":"link"}