anti-Chloride Channel 1, Skeletal Muscle (CLCN1) Antikörper

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. Zusätzlich bieten wir Ihnen Chloride Channel 1, Skeletal Muscle Kits (12) und Chloride Channel 1, Skeletal Muscle Proteine (7) und viele weitere Produktgruppen zu diesem Protein an.

Alle Antikörper anzeigen Gen GeneID UniProt
CLCN1 12723 Q64347
CLCN1 25688 P35524
CLCN1 1180 P35523
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Top anti-Chloride Channel 1, Skeletal Muscle Antikörper auf antikoerper-online.de

Showing 10 out of 45 products:

Katalog Nr. Reaktivität Wirt Konjugat Applikation Bilder Menge Anbieter Lieferzeit Preis Details
Rind (Kuh) Kaninchen Unkonjugiert WB WB Suggested Anti-Clcn1 Antibody Titration:  0.2-1 ug/ml  ELISA Titer:  1:62500  Positive Control:  Rat Brain Lanes:   1: 20ug mouse muscle membrane fraction, 2: Empty, 3: 20ug mouse brain membrane fraction, 4: Empty, 5: 20ug mouse muscle cytosolic fraction, 6: 20ug mouse brain cytosolic fraction  Primary Antibody Dilution:   1:1000  Secondary Antibody:   Anti-rabbit HRP  Secondary Antibody Dilution:   1:2000  Gene Name:   Clcn1  Submitted by:   Anonymous 100 μL Anmelden zum Anzeigen 2 bis 3 Tage
$289.00
Details
Human Kaninchen Unkonjugiert WB Western blot analysis of CLCN1 expression in Hela (A), NIH3T3 (B), H9C2 (C) whole cell lysates. 200 μL Anmelden zum Anzeigen 13 bis 14 Tage
$487.50
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Hund Kaninchen Unkonjugiert WB 50 μg Anmelden zum Anzeigen 11 bis 14 Tage
$551.83
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Human Kaninchen Unkonjugiert ELISA, WB Western blot analysis of CLCN1 using K562 whole cell lysates 100 μL Anmelden zum Anzeigen 11 bis 12 Tage
$390.77
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Human Kaninchen Unkonjugiert WB Western blot analysis of extracts of various cell lines, using CLCN1 antibody (ABIN5973365) at 1/1000 dilution. 100 μL Anmelden zum Anzeigen 11 bis 16 Tage
$426.40
Details
Maus Kaninchen Unkonjugiert IHC, WB Western Blot: CLCN1 Antibody [NBP1-69124] - Rat Brain lysate, concentration 0.2-1 ug/ml. Western Blot: CLCN1 Antibody [NBP1-69124] - Lanes: 1: 20 ug mouse muscle membrane fraction, 2: Empty, 3: 20 ug mouse brain membrane fraction, 4: Empty, 5: 20 ug mouse muscle cytosolic fraction, 6: 20 ug mouse brain cytosolic fraction Primary Antibody Dilution: 1:1000 Secondary Antibody: Anti-rabbit 100 μL Anmelden zum Anzeigen 8 bis 11 Tage
$488.73
Details
Human Kaninchen Unkonjugiert IHC, WB Western blot analysis of extracts of 293T and mouse liver cell lines, using CLCN1 antibody. 100 μL Anmelden zum Anzeigen 11 bis 13 Tage
$366.77
Details
Human Kaninchen Unkonjugiert WB Clcn1 antibody used at 5 ug/ml to detect target protein. 50 μg Anmelden zum Anzeigen 9 bis 11 Tage
$473.93
Details
Human Kaninchen Unkonjugiert WB Western blot analysis CLCN1 using K562 whole cell lysates. 100 μg Anmelden zum Anzeigen 11 bis 16 Tage
$454.83
Details
Human Kaninchen Unkonjugiert IHC, WB   100 μL Anmelden zum Anzeigen 7 bis 8 Tage
$454.67
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Weitere Antikörper gegen Chloride Channel 1, Skeletal Muscle Interaktionspartner

Mouse (Murine) Chloride Channel 1, Skeletal Muscle (CLCN1) Interaktionspartner

  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 (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, ClC-1, in Huntington disease 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 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 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

  11. alternative splicing is a posttranscriptional mechanism regulating chloride conductance during muscle development, and the chloride channelopathy in a transgenic mouse model of DM1 results from a failure to execute a splicing transition for CLCN1.

  12. These results support a molecular mechanism for myotonia in myotonic dystrophy type 1 in which a reduction in both the number of functional sarcolemmal ClC-1 and maximal channel open probability.

  13. Clc1 expression was dramatically decreased in Znf9+/- mice.

  14. genetic CLC-1 chloride channel deficiency in mice not only produces myotonia but also substantially worsens the isotonic contractile performance of diaphragm muscle.

  15. These observations indicate that the myotonia and chloride channelopathy observed in DM both result from abnormal alternative splicing of ClC-1

Human Chloride Channel 1, Skeletal Muscle (CLCN1) Interaktionspartner

  1. The distinction between CLC-0/1/2 channels and CLC transporters seems undetectable by amino acid sequence; to understand why they are different functionally, study determined the structure of the human CLC-1 channel. subtle differences in glutamate gate conformation, internal pore diameter and Cl(-) affinity distinguish CLC channels and transporters.

  2. FKBP8 Enhances Protein Stability of the CLC-1 Chloride Channel at the Plasma Membrane

  3. The novel missense CLCN1 mutations is associated with a family affected with myotonia congenital.

  4. Loss-of- function mutations in the CLCN1 gene located on 7q35, are the primary contributors to the pathogenesis of Thomsen myotonia

  5. Regulation of CLC-1 chloride channel biosynthesis by FKBP8 and Hsp90beta as a molecular model for myotonia congenita has been described.

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

  7. 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.

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

  9. 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

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

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

  12. Three novel mutations including 2 missense and one splicing were found in myotonia congenita patients.

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

  14. 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.

  15. Our data are consistent with the idea that the CUL4A/B-DDB1-CRBN complex catalyses the polyubiquitination and thus controls the degradation of CLC-1 channels.

  16. investigated sequences of PRRT2 and CLCN1 in a proband diagnosed with paroxysmal kinesigenic dyskinesia and suspected myotonia congenita; the proband and his father harbored a PRRT2 c.649dupC mutation, and CLCN1 c.1723C>T and c.2492A>G mutations; first report showing the coexistence of PRRT2 and CLCN1 mutations

  17. This electrophysiological and clinical observations suggest that heterozygous CLCN1 mutations can modify the clinical and electrophysiological expression of SCN4A mutation.

  18. splicing mutations accounted for 23 percent of all pathogenic variants in the cohort of myotonia congenita patients; 4 were heterozygous mutations in 4 unrelated individuals: c.563G>T in exon 5; c.1169-5T>G in intron 10; c.1251+1G>A in intron 11 and c.1931-2A>G in intron 16

  19. Six mutant forms of human CLC1 demonstrate modifications of channel gating behaviors and reduced chloride conductances that likely contribute to the physiologic changes of myotonia congenita.

  20. in Czech patients with myotonia congenita(MC), 34 differentCLCN1 mutations were identified in 51 MC probands (14 new mutations); structural analysis of mutations in the homology model of the dimeric ClC-1 protein was performed; results demonstrate structure-function relationships in the ClC-1 protein which are relevant to understanding the molecular pathogenesis of MC

Chloride Channel 1, Skeletal Muscle (CLCN1) Antigen-Profil

Protein Überblick

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.

Genbezeichner und Symbole assoziert mit CLCN1

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

Bezeichner auf Proteinebene für 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 SPEZIES
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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