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AG316 Acetylcholine Receptor-ε, control peptide for AB5938

Research Category: Neuroscience Research Sub-Category: Neurotransmitters & Receptors Gene Symbol: CHRNE, FCCMS, ACHRE, CMS1E, CMS1D, CMS2A, SCCMS UniProt Number: Q04844
AG316
100 µg  
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      Description
      Catalogue NumberAG316
      Brand Family Chemicon®
      Trade Name
      • Chemicon
      DescriptionAcetylcholine Receptor-ε, control peptide for AB5938
      OverviewPeptide from mouse acetylcholine receptor epsilon.
      References
      Product Information
      PresentationLiquid in PBS.
      Quality LevelMQ300
      Applications
      Key Applications
      • Cell Function Assay
      Application NotesFor use in blocking the reactivity of AB5938.

      Optimal working dilution must be determined by the end user.
      Biological Information
      Concentration1.0 mg/mL
      Entrez Gene Number
      Entrez Gene SummaryAcetylcholine receptors at mature mammalian neuromuscular junctions are pentameric protein complexes composed of four subunits in the ratio of two alpha subunits to one beta, one epsilon, and one delta subunit. The achetylcholine receptor changes subunit composition shortly after birth when the epsilon subunit replaces the gamma subunit seen in embryonic receptors. Mutations in the epsilon subunit are associated with congenital myasthenic syndrome.
      Gene Symbol
      • CHRNE
      • FCCMS
      • ACHRE
      • CMS1E
      • CMS1D
      • CMS2A
      • SCCMS
      UniProt Number
      UniProt SummaryFUNCTION: SwissProt: Q04844 # After binding acetylcholine, the AChR responds by an extensive change in conformation that affects all subunits and leads to opening of an ion-conducting channel across the plasma membrane.
      SIZE: 493 amino acids; 54697 Da
      SUBUNIT: Pentamer of two alpha chains, and one each of the beta, delta, and gamma (in immature muscle) or epsilon (in mature muscle) chains.
      SUBCELLULAR LOCATION: Cell junction, synapse, postsynaptic cell membrane; Multi-pass membrane protein.
      DISEASE: SwissProt: Q04844 # The muscle AChR is the major target antigen in the autoimmune disease myasthenia gravis [MIM:254200]. Myasthenia gravis is characterized by sporadic muscular fatigability and weakness, occurring chiefly in muscles innervated by cranial nerves, and characteristically improved by cholinesterase- inhibiting drugs. & Defects in CHRNE are a cause of slow-channel congenital myasthenic syndrome (SCCMS) [MIM:601462, 254200]. Congenital myasthenic syndromes (CMS) are inherited disorders of neuromuscular transmission that stem from mutations in presynaptic, synaptic, or postsynaptic proteins. Postsynaptic disorders result from mutations in proteins forming the subunits of the muscle acetylcholine receptor (AChR). The kinetic abnormalities of AChR result in either prolonged ion channel activations that underlie slow-channel myasthenic syndromes (SCCMS) or abbreviated channel activations that underlie the abnormally rapid decay of endplate currents in fast-channel syndromes (FCCMS). A third disorder associated with postsynaptic CMS is called CMS type Id (CMS1d), and could also result from mutations in the proteins forming the subunits of the muscle AChR. Mutations underlying SCCMS cause a gain of function and usually show dominant inheritance. & Defects in CHRNE are a cause of fast-channel congenital myasthenic syndrome (FCCMS) [MIM:608930]. Mutations underlying FCCMS cause a loss of function and show recessive inheritance. & Defects in CHRNE are a cause of congenital myasthenic syndrome type Id (CMS1d) [MIM:608931]; also called congenital myasthenic syndrome associated with acetylcholine receptor deficiency. Mutations underlying AChR deficiency cause a loss of function and show recessive inheritance.
      SIMILARITY: SwissProt: Q04844 ## Belongs to the ligand-gated ionic channel (TC 1.A.9) family.
      Physicochemical Information
      Dimensions
      Materials Information
      Toxicological Information
      Safety Information according to GHS
      Safety Information
      Product Usage Statements
      Usage Statement
      • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
      Storage and Shipping Information
      Storage ConditionsMaintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.
      Packaging Information
      Material Size100 µg
      Transport Information
      Supplemental Information
      Specifications
      Global Trade Item Number
      Catalogue Number GTIN
      AG316 04053252619038

      Documentation