Thermo Fisher Scientific CLCN1 Recombinant Rabbit Monoclonal Antibody (0P2M8)
Rabbit monoclonal antibody recognizing human CLCN1, validated for WB and ELISA applications. Recombinant expression in HEK293 cells ensures high specificity and reproducibility. Supplied as liquid, 1 mg/mL in PBS with BSA and glycerol. For research use...
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Applications
| Application | Tested Dilution |
|---|---|
| Western Blot (WB) | 1:2,000–1:20,000 |
| ELISA | 1 µg/mL |
Product Specifications
| Property | Description |
|---|---|
| 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 |
| Conjugate | 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.
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 it 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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