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Chloride Channel 1, Skeletal Muscle (CLCN1) ELISA Kits

The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. Additionally we are shipping Chloride Channel 1, Skeletal Muscle Antibodies (35) and Chloride Channel 1, Skeletal Muscle Proteins (7) and many more products for this protein.

list all ELISA KIts Gene Name GeneID UniProt
Anti-Mouse CLCN1 CLCN1 12723 Q64347
CLCN1 25688 P35524
CLCN1 1180 P35523
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Top Chloride Channel 1, Skeletal Muscle ELISA Kits at antibodies-online.com

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Human 0.1 ng/mL 0.5-10 ng/mL   96 Tests Log in to see 15 to 18 Days
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More ELISA Kits for Chloride Channel 1, Skeletal Muscle Interaction Partners

Mouse (Murine) Chloride Channel 1, Skeletal Muscle (CLCN1) interaction partners

  1. Data show that Muscleblind-like 1 (Mbnl1) and Muscleblind-Like 3 (Mbnl3) bind skeletal muscle chloride channel CIC-1 (Clc-1) mRNA.

  2. Sex hormones at high concentration can rapidly modulate ClC-1 in mouse skeletal muscle fibers in vitro.

  3. Myotonia (delayed muscle relaxation) is the most commonly observed symptom in DM1 patients and is caused by aberrant splicing of the skeletal muscle chloride channel (show CLCA1 ELISA Kits) (CLCN1) gene

  4. Myotonia in adult human skeletal actin transgenic mice may be explained on the basis of a mosaic expression of ClC-1 channels in different fibres and/or on alterations of other conductances.

  5. The expression of the muscle chloride channel (show CLCA1 ELISA Kits), ClC-1, in Huntington disease (show HTT ELISA Kits) muscle was compromised by improper splicing and a corresponding reduction in total Clcn1 (gene for ClC-1) mRNA.

  6. the majority of functional ClC-1 channels localize to the sarcolemma and provide essential insight into the basis of myofiber excitability in normal and diseased skeletal muscle.

  7. Expanded CUG repeats trigger aberrant splicing of ClC-1 chloride channel (show CLCA1 ELISA Kits) pre-mRNA and hyperexcitability of skeletal muscle in myotonic dystrophy

  8. The A331T mutation causes an unprecedented alteration of ClC-1 gating and reveals novel processes defining transitions between open and closed states in ClC (show CLC ELISA Kits) chloride channels

  9. A distinct autosomal recessive myotonic mouse in the C57BL/6 background (line B6MT)is reported in which the Clc-1 gene shows polymorphism with no functional consequences.

  10. CLC-1 deficiency not only affects muscle relaxation (myotonia) but also modulates diaphragm performance during the contractile phase of the contraction-relaxation cycle

Human Chloride Channel 1, Skeletal Muscle (CLCN1) interaction partners

  1. Regulation of CLC-1 chloride channel (show CLCA1 ELISA Kits) biosynthesis by FKBP8 (show FKBP8 ELISA Kits) and Hsp90beta (show HSP90AB1 ELISA Kits) as a molecular model for myotonia congenita has been described.

  2. the spectrum of CLCN1 mutations in patients with Myotonia Congenita

  3. Combining our results with the literature on Chinese populations indicates that 21 mutations in CLCN1 have been associated with myotonia congenital, while 7 mutations in SCN4A have been associated with paramyotonia congenita, 2 mutations in SCN4A have been associated with sodium channel myotonias.

  4. report a novel ClC-1 mutation, T335N, that is associated with a mild phenotype

  5. The present study is the first demonstration of ClC-1 regulation in active human muscle, and it provides a detailed description of the involvement of PKC and ClC-1 in the down-regulation of Gm during AP-firing activity in human skeletal muscle fibres

  6. This study, novel mutations in CLCN1 were detected, and the spectrum of CLCN1 mutations known to be associated with MC was expanded.

  7. our study confirms the presence of the myotonia causing CLCN1 mutations p.F167L and p.R105C in the Costa Rican population.

  8. we characterized three other myotonic ClC-1 mutations.

  9. In 4 patients (3 families) with recessive MC, 4 CLCN1 variants were found, 3 of which are new. c.244A>G (p.T82A) and c.1357C>T (p.R453W) were compound heterozygotes with c.568GG>TC (p.G190S). The new c.809G>T (p.G270V) was homozygous.

  10. Our data are consistent with the idea that the CUL4A/B-DDB1-CRBN (show CRBN ELISA Kits) complex catalyses the polyubiquitination and thus controls the degradation of CLC-1 channels.

Chloride Channel 1, Skeletal Muscle (CLCN1) Antigen Profile

Antigen Summary

The CLCN family of voltage-dependent chloride channel genes comprises nine members (CLCN1-7, Ka and Kb) which demonstrate quite diverse functional characteristics while sharing significant sequence homology. The protein encoded by this gene regulates the electric excitability of the skeletal muscle membrane. Mutations in this gene cause two forms of inherited human muscle disorders: recessive generalized myotonia congenita (Becker) and dominant myotonia (Thomsen). Alternative splicing results in multiple transcript variants.

Gene names and symbols associated with CLCN1

  • chloride voltage-gated channel 1 (CLCN1) antibody
  • chloride channel, voltage-sensitive 1a (clcn1a) antibody
  • chloride channel protein 1 (LOC100550479) antibody
  • chloride channel protein 1 (LOC703944) antibody
  • chloride channel, voltage-sensitive 1 (Clcn1) antibody
  • chloride voltage-gated channel 1 (Clcn1) antibody
  • adr antibody
  • Clc-1 antibody
  • CLC1 antibody
  • CLCN1 antibody
  • mto antibody
  • myotonia antibody
  • nmf355 antibody
  • si:dkey-14o18.5 antibody
  • SMCC antibody
  • SMCC1 antibody

Protein level used designations for CLCN1

chloride channel 1, skeletal muscle , chloride channel 1, skeletal muscle (Thomsen disease, autosomal dominant) , chloride channel protein 1-like , similar to chloride channel 1, skeletal muscle (Thomsen disease, autosomal dominant) , arrested development of righting response , chloride channel protein 1 , chloride channel protein, skeletal muscle , chloride channel 1, skeletal muscle C-type , clC-1 , skeletal muscle chloride channel 1 , skeletal muscle chloride channel ClC-1

GENE ID SPECIES
100011971 Monodelphis domestica
100050692 Equus caballus
100349073 Oryctolagus cuniculus
100402136 Callithrix jacchus
472560 Pan troglodytes
795133 Danio rerio
100550479 Meleagris gallopavo
100601183 Nomascus leucogenys
703944 Macaca mulatta
12723 Mus musculus
514597 Bos taurus
25688 Rattus norvegicus
1180 Homo sapiens
403723 Canis lupus familiaris
427949 Gallus gallus
100733719 Cavia porcellus
100520812 Sus scrofa
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