CHRND抗体;CHRND Polyclonal Antib

Description The acetylcholine receptor of muscle has 5 subunits of 4 different types: 2 alpha and 1 each of beta, gamma and delta subunits. After acetylcholine binding, the receptor undergoes an extensive conformation change that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane. Defects in this gene are a cause of multiple pterygium syndrome lethal type (MUPSL), congenital myasthenic syndrome slow-channel type (SCCMS), and congenital myasthenic syndrome fast-channel type (FCCMS). Several transcript variants encoding different isoforms have been found for this gene. References 1.Clin Dysmorphol. 2013 Apr;22(2):54-8. 2.J Biol Chem. 2014 Jan 3;289(1):203-14. Specification Aliases:ACHRD; CMS2A; CMS3A; CMS3B; CMS3C; FCCMS; SCCMS Entrez GeneID:1144 Swissprot:Q07001 clone:1H1F9 WB Predicted band size:58.8kDa Host/Isotype:Mouse IgG1 Antibody Type:Primary antibody Storage:Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. Species Reactivity:Human Immunogen:Purified recombinant fragment of human CHRND (AA: extra 22-245) expressed in E. Coli. Formulation:Purified antibody in PBS with 0.05% sodium azide Application WB:1/500 - 1/2000 FCM:1/200 - 1/400 ELISA:1/10000