货号：AB5938

品牌：Merck Millipore

规格：EA

目录价：询价

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Acetylcholine Receptor-ε Antibody | AB5938 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Acetylcholine Receptor epsilon. View All» Immunogen: Synthetic peptide. View All» Species Reactivity: Mouse View All» Application Notes: Optimal working dilutions must be determined by end user. View All» Presentation: Affinity purified chicken IgY. Liquid. View All» Storage Conditions: Maintain at 2-8°C in undiluted aliquots for up to 6 months after date of receipt. View All» UniProt Number: Q04844 View All» Entrez Gene Number: NM_000080.2 View All» Gene Symbol:

• CHRNE

• FCCMS

• ACHRE

• CMS1E

• CMS1D

• CMS2A

• SCCMS
  View All» 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. View All» Key Applications: Immunohistochemistry View All» Entrez Gene Summary: Acetylcholine 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. View All» UniProt Summary: FUNCTION: 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. View All» Brand Family: Chemicon View All» Product Name: Anti-Acetylcholine Receptor-ε Antibody | AB5938 View All» Antibody Type: Polyclonal Antibody View All» Qty/Pk: 100 µg View All» Format: Affinity Purified View All» Host: Chicken View All»
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