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Alomone Labs, LTD
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Reconstitution: 50 µL or 0.2 mL double distilled water (DDW), depending on the sample size. The antibody ships as a lyophilized powder at room temperature. Upon arrival, it should be stored at -20C. The reconstituted solution can be stored at 4C for up to 1 week. For longer periods, small aliquots should be stored at -20C. Avoid multiple freezing and thawing. Centrifuge all antibody preparations before use (10000 x g 5 min).
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: arrested development of righting response; chloride channel 1, skeletal muscle; Chloride channel protein 1; Chloride channel protein, skeletal muscle; CIC 1; CIC1; ClC-1; MGC138361; MGC142055
基因别名: adr; Clc-1; Clc1; Clcn1; mto; myotonia; nmf355; SMCC; SMCC1
UniProt ID: (Mouse) Q64347, (Rat) P35524
Entrez Gene ID: (Mouse) 12723, (Rat) 25688