Anti-CLCN1 antibody

Catalog Number:

A001036412STJ

Mfr. No.:

STJ28306

Price:

$297

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Overview
- The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). Alternative splicing results in multiple transcript variants.
  
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Properties
- Host
  
  rabbit
  
  Antibody Type
  
  polyclonal
  
  Isotype
  
  IgG
  
  Reactivity
  
  human; mouse
  
  Immunogen
  
  Recombinant fusion protein containing a sequence corresponding to amino acids 779-988 of human CLCN1 (NP_000074. 2).
  
  Purification
  
  Affinity purification
  
  Conjugate
  
  Unconjugated
  
  Formulation
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  
  Storage
  
  Store at -20°C and avoid freeze-thaw cycles.
  
  Regulatory Status
  
  RUO
  
  Antigen
  
  Chloride Channel Protein 1; Clc-1; Chloride Channel Protein-Skeletal Muscle (Gene ID:1180; UniProt:P35523)
  
  * For research use only