Invitrogen™ CLCN1 Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA537147

Catalog No. PIPA537147

Description

This antibody detects endogenous protein at a molecular weight of 110 kDa. Purity is >95% by SDS-PAGE.

The family of voltage-dependent chloride channels (CLCs) regulate cellular trafficking of chloride ions, a critical component of all living cells. CLCs regulate excitability in muscle and nerve cells, aid in organic solute transport and maintain cellular volume. CLC1 is highly expressed in skeletal muscle. Mutations in the gene encoding CLC1 lead to myotonia, an inheritable disorder characterized by muscle stiffness and renal salt wasting. CLC2 is highly expressed in the epithelia of several organs including lung, which suggests CLC2 may be a possible therapeutic target for cystic fibrosis. CLC3 expression is particularly abundant in neuronal tissue, while CLC4 expression is evident in skeletal and cardiac muscle as well as brain. Mutations in the gene encoding CLC5 lead to Dent's disease, a renal disorder characterized by proteinuria and hypercalciuria. CLC6 and CLC7 are broadly expressed in several tissues including testis, kidney, brain and muscle.

Specifications

• Antigen: CLCN1
• Classification: Polyclonal
• Conjugate: Unconjugated
• Gene: CLCN1
• Gene Alias: adr; arrested development of righting response; chloride channel 1; chloride channel 1, skeletal muscle; chloride channel protein 1; Chloride channel protein, skeletal muscle; chloride channel, voltage-sensitive 1; chloride voltage-gated channel 1; CIC 1; CIC1; CLC1; Clc-1; Clcn1; MGC138361; MGC142055; mto; myotonia; nmf355; SMCC; SMCC1
• Host Species: Rabbit
• Purification Method: Antigen Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 1180, 12723, 25688
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Western Blot
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide
• Gene Accession No.: P35523, P35524, Q64347
• Gene Symbols: CLCN1
• Immunogen: A synthetic peptide corresponding to residues in human CLCN1.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG