{"product_id":"du4l7-rabbit-polyclonal-antibody-es16882","title":"DU4L7 Rabbit Polyclonal Antibody - ES16882","description":"\u003cp\u003eDU4L7 Rabbit Polyclonal Antibody\u003c\/p\u003e\n\n\u003cp\u003eSizes: 50μL, 100μL\u003c\/p\u003e\n\n\u003cp\u003eCatalogue Numbers: ES16882-50, ES16882-100\u003c\/p\u003e\n\n\u003cp\u003eLead times: 1-2 weeks, if manufacturer has product in stock\u003c\/p\u003e\n\n\u003cp\u003eManufacturer\/Ship Location: China\u003c\/p\u003e\n\n\u003cp\u003eBackground: This gene is located within a D4Z4 repeat array in the subtelomeric region of chromosome 4q. The D4Z4 repeat is polymorphic in length and a similar D4Z4 repeat array has been identified on chromosome 10. Each D4Z4 repeat unit has an open reading frame (named DUX4) that encodes two homeoboxes, the repeat-array and ORF is conserved in other mammals. There is no evidence for transcription of the gene at this locus though RT-PCR and in vitro expression experiments indicate that a telomeric paralog of this gene is transcribed in some haplotypes. Contraction of the macrosatellite repeat causes autosomal dominant facioscapulohumeral muscular dystrophy (FSHD). [provided by RefSeq, Jun 2014],\u003c\/p\u003e\n\n\u003cp\u003eReactivity: Human, Rat, Mouse,\u003c\/p\u003e \n\n\u003cp\u003eHost: Rabbit\u003c\/p\u003e\n\n\u003cp\u003eApplications: WB\u003c\/p\u003e\n\n\u003cp\u003eClonality: Polyclonal\u003c\/p\u003e\n\n\u003cp\u003eImmunogen: Synthesized peptide derived from human DU4L7 AA range: 314-364\u003c\/p\u003e\n\n\u003cp\u003eIsotype: IgG\u003c\/p\u003e\n\n\u003cp\u003eTarget: DU4L7\u003c\/p\u003e\n\n\u003cp\u003eSpecificity: This antibody detects endogenous levels of DU4L7 at Human\u003c\/p\u003e\n\n\u003cp\u003eDilution: WB 1：500-2000\u003c\/p\u003e\n\n\u003cp\u003ePurification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.\u003c\/p\u003e\n\n\u003cp\u003eConcentration: 1 mg\/ml,\u003c\/p\u003e\n\n\u003cp\u003eFormlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.\u003c\/p\u003e\n\n\u003cp\u003eMolecular Weight (Da): 47kD\u003c\/p\u003e\n\n\u003cp\u003eFunction: Disease: Defects in DUX4 may be the cause of facioscapulohumeral muscular dystrophy (FSHD) [MIM: 158900]. FSHD is characterized by weakness of the muscles of the face, upper-arm and shoulder girdle. Severity is highly variable. Weakness is slowly progressive and about 20% of affected individuals eventually require a wheelchair. Approximately 70-90% of individuals have inherited the disease-causing deletion from a parent, and approximately 10-30% of affected individuals have FSHD as the result of a de novo deletion. Offsprings of an affected individual have a 50% chance of inheriting the deletion\u003cbr\u003e\nDomain: Both homebox domains confer nuclear targeting\u003cbr\u003e\nFunction: May be involved in transcriptional regulation\u003cbr\u003e\nMiscellaneous: DUX genes are present in 3.3-kilobase elements, a tandem repeat family scattered in the genome found on the short arms of all acrocentric chromosomes as well as on several ot\u003c\/p\u003e\n\n\u003cp\u003eSubcellular Location: Nucleus\u003c\/p\u003e\n\n\u003cp\u003eStorage and Stability: -20°C\/1 year\u003c\/p\u003e\n\n\u003cp\u003eGene Name: DUX4L7\u003c\/p\u003e\n\n\u003cp\u003eProtein Name: DU4L7\u003c\/p\u003e\n\n\u003cp\u003eResearch Use Only\u003c\/p\u003e","brand":"ELK Biotechnology","offers":[{"title":"50μL","offer_id":46315487789235,"sku":"ES16882-50","price":186.0,"currency_code":"CAD","in_stock":true},{"title":"100μL","offer_id":46315487822003,"sku":"ES16882-100","price":372.0,"currency_code":"CAD","in_stock":true}],"thumbnail_url":"\/\/cdn.shopify.com\/s\/files\/1\/0305\/9482\/6376\/files\/elk_antibodies.jpg?v=1793190497","url":"https:\/\/afsbio.com\/products\/du4l7-rabbit-polyclonal-antibody-es16882","provider":"AFSBio Inc.","version":"1.0","type":"link"}