Thermo Fisher Scientific CLCN1 Recombinant Rabbit Monoclonal Antibody (0P2M8)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:2,000-1:20,000

-

ELISA (ELISA)

1 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

0P2M8

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 889-988 of human CLCN1. if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:CLCN1,uniProtId:P35523-1,ncbiNodeId:9606,antigenRange:889-988,antigenLength:988,antigenImageFileName:MA5-55411_CLCN1_P35523-1_Rabbit.svg,antigenImageFileNamePDP:MA5-55411_CLCN1_P35523-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Protein A

Storage buffer

PBS, pH 7.3, with 0.05% BSA, 50% glycerol

Contains

0.05% ProClin 300

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Product Specific Information

Immunogen Sequence: NTTSTRKSTG APPSSAENWN LPEDRPGATG TGDVIAASPE TPVPSPSPEP PLSLAPGKVE GELEELELVE SPGLEEELAD ILQGPSLRST DEEDEDELIL

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. 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.

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