Thermo Fisher Scientific CLCN1 Polyclonal Antibody

Applications

Western Blot (WB)

• Tested Dilution: 1:1,000–1:3,000

Product Specifications

Product Specific Information

Antibody detects endogenous levels of total CLCN1.

Target Information

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.
CLCN1 is highly expressed in skeletal muscle. Mutations in the gene encoding CLCN1 lead to myotonia, an inheritable disorder characterized by muscle stiffness and renal salt wasting.
CLCN2 is highly expressed in the epithelia of several organs including lung, suggesting CLCN2 may be a possible therapeutic target for cystic fibrosis.
CLCN3 expression is particularly abundant in neuronal tissue, while CLCN4 expression is evident in skeletal and cardiac muscle as well as brain.
Mutations in the gene encoding CLCN5 lead to Dent’s disease, a renal disorder characterized by proteinuria and hypercalciuria.
CLCN6 and CLCN7 are broadly expressed in several tissues including testis, kidney, brain, and muscle.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.

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