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Anti-CHRNB1 Antibody

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产品名称: Anti-CHRNB1 Antibody
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Anti-CHRNB1 Antibody


Anti-CHRNB1 Antibody  的详细介绍
Name: Anti-CHRNB1 Antibody
See all CHRNB1 primary antibodies
Description: Goat polyclonal antibody to CHRNB1.
Applications: ELISA, WB
Reactivity: Human
Immunogen: Synthetic peptide corresponding to Human CHRNB1 (internal region).
Sequence: C-QEQEDHDALKED
Host: Goat
Clonality: Polyclonal
Isotype: IgG
Conjugate: Unconjugated
Purification: Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide.
Concentration: 100 µg at 0.5 mg/ml.
Product Form: Liquid
Formulation: Supplied in Tris Buffered Saline, pH 7.30, with 0.02% Sodium Azide and 0.5% BSA.
Storage: Shipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Function: 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.
Involvement in Disease: Myasthenic syndrome, congenital, 2A, slow-channel: A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. CMS2A is a slow-channel myasthenic syndrome. It is caused by kinetic abnormalities of the AChR, resulting in prolonged AChR channel opening episodes, prolonged endplate currents, and depolarization block. This is associated with calcium overload, which may contribute to subsequent degeneration of the endplate and postsynaptic membrane.

Myasthenic syndrome, congenital, 2C, associated with acetylcholine receptor deficiency: A form of congenital myasthenic syndrome, a group of disorders characterized by failure of neuromuscular transmission, including pre-synaptic, synaptic, and post-synaptic disorders that are not of autoimmune origin. Clinical features are easy fatigability and muscle weakness affecting the axial and limb muscles (with hypotonia in early-onset forms), the ocular muscles (leading to ptosis and ophthalmoplegia), and the facial and bulbar musculature (affecting sucking and swallowing, and leading to dysphonia). The symptoms fluctuate and worsen with physical effort. CMS2C is an autosomal recessive disorder of postsynaptic neuromuscular transmission, due to deficiency of AChR at the endplate that results in low amplitude of the miniature endplate potential and current. CMS2C is clinically characterized by early-onset muscle weakness with variable severity.
Sequence Similarities: Belongs to the ligand-gated ion channel (TC 1.A.9) family. Acetylcholine receptor (TC 1.A.9.1) subfamily. Beta-1/CHRNB1 sub-subfamily.
Cellular Location: Cell junction > Synapse > Postsynaptic cell membrane. Cell membrane.
Database Links:
  • Entrez Gene: 1140 Human
  • GenBank: NP_000738.2 Human
  • Omim: 100710 Human
  • SwissProt: P11230 Human
  • Unigene: 330386 Human
    		•
    Synonyms:
  • Acetylcholine receptor protein beta chain precursor Antibody
  • Acetylcholine receptor subunit beta Antibody
  • ACHB_HUMAN Antibody
  • AChR Antibody
  • ACHRB Antibody
  • Cholinergic receptor nicotinic beta polypeptide 1 (muscle) Antibody
  • CHRNB Antibody
  • Chrnb 1 Antibody
  • chrnb1 Antibody
  • CMS1D Antibody
  • CMS2A Antibody
  • Nicotinic Acetylcholine Receptor beta Antibody
  • Nicotinic acetylcholine receptor beta subunit precursor Antibody
  • SCCMS Antibody
    		•
    Information: Target information shown above is from the UniProt Consortium.
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