Details for: CLCN1

Gene ID: 1180

Symbol: CLCN1

Ensembl ID: ENSG00000188037

Description: chloride voltage-gated channel 1

Associated with

Other Information

Genular Protein ID: 113988378

Symbol: CLCN1_HUMAN

Name: Chloride channel protein 1

UniProtKB Accession Codes:

Database IDs:

Citations:

PubMed ID: 8112288

Title: Multimeric structure of ClC-1 chloride channel revealed by mutations in dominant myotonia congenita (Thomsen).

PubMed ID: 8112288

DOI: 10.1002/j.1460-2075.1994.tb06315.x

PubMed ID: 12690205

Title: Human chromosome 7: DNA sequence and biology.

PubMed ID: 12690205

DOI: 10.1126/science.1083423

PubMed ID: 15489334

Title: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).

PubMed ID: 15489334

DOI: 10.1101/gr.2596504

PubMed ID: 1379744

Title: The skeletal muscle chloride channel in dominant and recessive human myotonia.

PubMed ID: 1379744

DOI: 10.1126/science.1379744

PubMed ID: 7981750

Title: Molecular basis of Thomsen's disease (autosomal dominant myotonia congenita).

PubMed ID: 7981750

DOI: 10.1038/ng0493-305

PubMed ID: 9122265

Title: A mutation in autosomal dominant myotonia congenita affects pore properties of the muscle chloride channel.

PubMed ID: 9122265

DOI: 10.1073/pnas.94.6.2729

PubMed ID: 12456816

Title: A novel alteration of muscle chloride channel gating in myotonia levior.

PubMed ID: 12456816

DOI: 10.1113/jphysiol.2002.027037

PubMed ID: 29809153

Title: Structure of the CLC-1 chloride channel from Homo sapiens.

PubMed ID: 29809153

DOI: 10.7554/elife.36629

PubMed ID: 7951242

Title: Genomic organization of the human muscle chloride channel ClC-1 and analysis of novel mutations leading to Becker-type myotonia.

PubMed ID: 7951242

DOI: 10.1093/hmg/3.6.941

PubMed ID: 7981681

Title: Proof of a non-functional muscle chloride channel in recessive myotonia congenita (Becker) by detection of a 4 base pair deletion.

PubMed ID: 7981681

DOI: 10.1093/hmg/3.7.1123

PubMed ID: 7874130

Title: Nonsense and missense mutations of the muscle chloride channel gene in patients with myotonia congenita.

PubMed ID: 7874130

PubMed ID: 8533761

Title: Spectrum of mutations in the major human skeletal muscle chloride channel gene (CLCN1) leading to myotonia.

PubMed ID: 8533761

PubMed ID: 7581380

Title: Myotonia levior is a chloride channel disorder.

PubMed ID: 7581380

DOI: 10.1093/hmg/4.8.1397

PubMed ID: 8845168

Title: Mutations in dominant human myotonia congenita drastically alter the voltage dependence of the CIC-1 chloride channel.

PubMed ID: 8845168

DOI: 10.1016/0896-6273(95)90023-3

PubMed ID: 8571958

Title: Novel muscle chloride channel mutations and their effects on heterozygous carriers.

PubMed ID: 8571958

PubMed ID: 9736777

Title: ClC-1 chloride channel mutations in myotonia congenita: variable penetrance of mutations shifting the voltage dependence.

PubMed ID: 9736777

DOI: 10.1093/hmg/7.11.1753

PubMed ID: 10215406

Title: Identification of five new mutations and three novel polymorphisms in the muscle chloride channel gene (CLCN1) in 20 Italian patients with dominant and recessive myotonia congenita.

PubMed ID: 10215406

DOI: 10.1002/(sici)1098-1004(1998)11:4<331::aid-humu12>3.0.co;2-3

PubMed ID: 9566422

Title: Novel muscle chloride channel (CLCN1) mutations in myotonia congenita with various modes of inheritance including incomplete dominance and penetrance.

PubMed ID: 9566422

DOI: 10.1212/wnl.50.4.1176

PubMed ID: 10644771

Title: Mechanism of inverted activation of ClC-1 channels caused by a novel myotonia congenita mutation.

PubMed ID: 10644771

DOI: 10.1074/jbc.275.4.2999

PubMed ID: 11113225

Title: A 'dystrophic' variant of autosomal recessive myotonia congenita caused by novel mutations in the CLCN1 gene.

PubMed ID: 11113225

DOI: 10.1212/wnl.55.11.1697

PubMed ID: 12661046

Title: Decrement of compound muscle action potential is related to mutation type in myotonia congenita.

PubMed ID: 12661046

DOI: 10.1002/mus.10347

PubMed ID: 16959974

Title: The consensus coding sequences of human breast and colorectal cancers.

PubMed ID: 16959974

DOI: 10.1126/science.1133427

PubMed ID: 19697366

Title: Myotonia congenita in a large consanguineous Arab family: insight into the clinical spectrum of carriers and double heterozygotes of a novel mutation in the chloride channel CLCN1 gene.

PubMed ID: 19697366

DOI: 10.1002/mus.21525

PubMed ID: 22521272

Title: Myotonia congenita: novel mutations in CLCN1 gene and functional characterizations in Italian patients.

PubMed ID: 22521272

DOI: 10.1016/j.jns.2012.03.024

PubMed ID: 22641783

Title: Disease-causing mutations C277R and C277Y modify gating of human ClC-1 chloride channels in myotonia congenita.

PubMed ID: 22641783

DOI: 10.1113/jphysiol.2012.232785

PubMed ID: 26096614

Title: ClC-1 mutations in myotonia congenita patients: insights into molecular gating mechanisms and genotype-phenotype correlation.

PubMed ID: 26096614

DOI: 10.1113/jp270358

PubMed ID: 26007199

Title: Clinical, molecular, and functional characterization of CLCN1 mutations in three families with recessive myotonia congenita.

PubMed ID: 26007199

DOI: 10.1007/s12017-015-8356-8

PubMed ID: 26502825

Title: Impaired surface membrane insertion of homo- and heterodimeric human muscle chloride channels carrying amino-terminal myotonia-causing mutations.

PubMed ID: 26502825

DOI: 10.1038/srep15382

PubMed ID: 26510092

Title: Identification and functional characterization of CLCN1 mutations found in nondystrophic myotonia patients.

PubMed ID: 26510092

DOI: 10.1002/humu.22916

PubMed ID: 27653901

Title: A case of non-dystrophic myotonia with concomitant mutations in the SCN4A and CLCN1 genes.

PubMed ID: 27653901

DOI: 10.1016/j.jns.2016.08.030

PubMed ID: 27666773

Title: Thomsen disease with ptosis and abnormal MR findings.

PubMed ID: 27666773

DOI: 10.1016/j.nmd.2016.08.016

Sequence Information:

  • Length: 988
  • Mass: 108626
  • Checksum: CA838BCD2AF3CA68
  • Sequence:
  • MEQSRSQQRG GEQSWWGSDP QYQYMPFEHC TSYGLPSENG GLQHRLRKDA GPRHNVHPTQ 
    IYGHHKEQFS DREQDIGMPK KTGSSSTVDS KDEDHYSKCQ DCIHRLGQVV RRKLGEDGIF 
    LVLLGLLMAL VSWSMDYVSA KSLQAYKWSY AQMQPSLPLQ FLVWVTFPLV LILFSALFCH 
    LISPQAVGSG IPEMKTILRG VVLKEYLTMK AFVAKVVALT AGLGSGIPVG KEGPFVHIAS 
    ICAAVLSKFM SVFCGVYEQP YYYSDILTVG CAVGVGCCFG TPLGGVLFSI EVTSTYFAVR 
    NYWRGFFAAT FSAFVFRVLA VWNKDAVTIT ALFRTNFRMD FPFDLKELPA FAAIGICCGL 
    LGAVFVYLHR QVMLGVRKHK ALSQFLAKHR LLYPGIVTFV IASFTFPPGM GQFMAGELMP 
    REAISTLFDN NTWVKHAGDP ESLGQSAVWI HPRVNVVIII FLFFVMKFWM SIVATTMPIP 
    CGGFMPVFVL GAAFGRLVGE IMAMLFPDGI LFDDIIYKIL PGGYAVIGAA ALTGAVSHTV 
    STAVICFELT GQIAHILPMM VAVILANMVA QSLQPSLYDS IIQVKKLPYL PDLGWNQLSK 
    YTIFVEDIMV RDVKFVSASY TYGELRTLLQ TTTVKTLPLV DSKDSMILLG SVERSELQAL 
    LQRHLCPERR LRAAQEMARK LSELPYDGKA RLAGEGLPGA PPGRPESFAF VDEDEDEDLS 
    GKSELPPSLA LHPSTTAPLS PEEPNGPLPG HKQQPEAPEP AGQRPSIFQS LLHCLLGRAR 
    PTKKKTTQDS TDLVDNMSPE EIEAWEQEQL SQPVCFDSCC IDQSPFQLVE QTTLHKTHTL 
    FSLLGLHLAY VTSMGKLRGV LALEELQKAI EGHTKSGVQL RPPLASFRNT TSTRKSTGAP 
    PSSAENWNLP EDRPGATGTG DVIAASPETP VPSPSPEPPL SLAPGKVEGE LEELELVESP 
    GLEEELADIL QGPSLRSTDE EDEDELIL

Database document:

This is a preview of the gene's schema. Only a few entries are kept for 'singleCellExpressions,' 'mRNAExpressions,' and other large data arrays for visualization purposes. You can zoom in with the mouse wheel for a closer view, and the text will adjust automatically if necessary. For the full schema, download it here.