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CHRND

Order Information
Catalog# Size/Concentration Price
31349 1.0mg/ml $341.00
Specification
Entrez GeneID 1144
Aliases ACHRD; CMS2A; CMS3A; CMS3B; CMS3C; FCCMS; SCCMS
Clone# 1H1F9
MW 58.8kDa
Host/Isotype Mouse IgG1
Storage 4℃; -20℃ for long term storage
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
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.
Sequence
extra(22-245)
Related Products

Elisa

Figure 1:Black line: Control Antigen (100 ng);Purple line: Antigen (10ng); Blue line: Antigen (50 ng); Red line:Antigen (100 ng)

Western Blot

Figure 2:Western blot analysis using CHRND mAb against human CHRND (AA: extra 22-245) recombinant protein. (Expected MW is 52.2 kDa)

Western Blot

Figure 3:Western blot analysis using CHRND mAb against HEK293 (1) and CHRND (AA: extra 22-245)-hIgGFc transfected HEK293 (2) cell lysate.

Western Blot

Figure 4:Western blot analysis using CHRND mouse mAb against C6 (1) cell lysate.

Flow cytometric

Figure 5:Flow cytometric analysis of SK-N-SH cells using CHRND mouse mAb (green) and negative control (red).