Clcn1
entrez
12723
canonical
uniprot
Q64347
canonical
ensembl
ENSMUSG00000029862
resolver
ensp
ENSMUSP00000031894
resolver
enst
ENSMUST00000031894
resolver
genesymbol
Clcn1
resolver
genesymbol_syn
Clc1
resolver
uniprot
Q8BZ41
resolver
uniprot
Q9QVY5
resolver
uniprot_entry
CLCN1_MOUSE
resolver
uniprot_sec
Q8BZ41
resolver
uniprot_sec
Q9QVY5
resolver
activity_regulation
ACTIVITY REGULATION: Modulated by membrane voltage with depolarization favouring channel opening and hyperpolarization favouring channel closure. Inhibited by acidic pH and ATP binding due to a shift of voltage dependence of common gating to more positive voltages. Inhibited by 9-anthracene-carboxylic. {ECO:0000250|UniProtKB:P35523}.
uniprot
proteins
amino_acid_sequence
MERSQSQRHGGEQSWWGSAPQYQYMPFEHCTSYGLPSENGGLQHRPRKDMGPRHNAHPTQIYGHQKEQYSYKAQDGGMPKKMGSSSTMDSLDEDHYSKCQDCVHRLGRVLRRKLGEDWIFLVLLGLLMALVSWCMDYVSAKSLQAYKWTYAQMKPSLPLQYLAWVTFPLILILFSALFCQLISPQAVGSGIPEMKTILRGVVLKEYLTLKAFVAKVVALTAGLGSGIPVGKEGPFVHIASICAAVLSKFMSMFSGVYEQPYYYTDILTVGCAVGVGCCFGTPLGGVLFSIEVTSTYFAVRNYWRGFFAATFSAFVFRVLAVWNKDAVTITALFRTNFRMDFPFDLKELPAFAVIGICCGFLGAVFVYLHRQVMLGVRKHKCLSQFLAKHRLLYPGIVTFVIASLTFPPGMGQFMAGELMPREAISTLFDNNTWVKHIGDPQSLGQSAVWLHPQVNVIIIILLFFVMKFWMSIVATTMPIPCGGFMPVFVLGAAFGRLVGEIMAMLFPEGILFDDIIYKILPGGYAVIGAAALTGAVSHTVSTAVICFELTGQIAHILPMMVAVILANMVAQSLQPSLYDSIIQVKKLPYLPDLGWNQLSKFTIFVEDIMVRDVKFVSASCTYGELRNLLQATTVKTLPLVDSKDSMILLGSVERSELQSLLQRHLCAERRLKAAQDMARKLSELPYNGKAQLAGDWHPGGRPESFAFVDEDEDEDLSRKMELPLTPAPPPPSPPPPPSQFPIAPSNPEEPNGPLPSHKQPPEASDSADQRSSTFQRLLHCLLGKAHSKKKKITQDSTDLVDNMSPEEIEAWEREQLSQPVCFDCCCIDQSPFQLVEQTTLHKTHTLFSLLGLHLAYVTSMGKLRGVLALEELQKAIEGHTKSGVQLRPPLASFRNTTSIRKTPGGPPPPAEGWNVPEDGDGAPGREVMVPTMPETPVPPPSPEAPSCLAPARAEGELEELEMVGSLEPEEELADILHGPSLRSTDEEDEDELIL
uniprot
proteins
function
FUNCTION: Voltage-gated chloride channel involved in skeletal muscle excitability. Generates most of the plasma membrane chloride conductance in skeletal muscle fibers, stabilizes the resting membrane potential and contributes to the repolarization phase during action potential firing (By similarity) (PubMed:18008009, PubMed:21078869, PubMed:21149546). Forms a homodimeric channel where each subunit has its own ion conduction pathway. Conducts double-barreled currents controlled by two types of gates, two fast glutamate gates that control each subunit independently and a slow common gate that opens and shuts off both subunits simultaneously (By similarity). Has a significant open probability at muscle resting potential and is further activated upon membrane depolarization. Permeable to small monovalent anions with ion selectivity for chloride > thiocyanate > bromide > nitrate > iodide (By similarity). {ECO:0000250|UniProtKB:P35523, ECO:0000269|PubMed:18008009, ECO:0000269|PubMed:21078869, ECO:0000269|PubMed:21149546}.
uniprot
proteins
mass_dalton
109794
uniprot
proteins
mutagenesis
MUTAGEN 553; /note="I->T: Causes myotonia."; /evidence="ECO:0000269|PubMed:8119941
uniprot
proteins
protein_family
Chloride channel (TC 2.A.49) family
uniprot
proteins
protein_family
ClC-1/CLCN1 subfamily
uniprot
proteins
pubmed
15489334
uniprot
proteins
pubmed
16141072
uniprot
proteins
pubmed
1659665
uniprot
proteins
pubmed
18008009
uniprot
proteins
pubmed
21078869
uniprot
proteins
pubmed
21149546
uniprot
proteins
pubmed
21183079
uniprot
proteins
pubmed
8119941
uniprot
proteins
pubmed
9321463
uniprot
proteins
sequence_length
994
uniprot
proteins
subcellular_location
SUBCELLULAR LOCATION: Cell membrane {ECO:0000250|UniProtKB:P35523}; Multi-pass membrane protein {ECO:0000250|UniProtKB:P35523}. Cell membrane, sarcolemma {ECO:0000269|PubMed:18008009, ECO:0000269|PubMed:21078869, ECO:0000305|PubMed:21149546}; Multi-pass membrane protein {ECO:0000255}. Cell membrane, sarcolemma, T-tubule {ECO:0000305|PubMed:21149546}; Multi-pass membrane protein {ECO:0000255}.
uniprot
proteins
transmembrane_region
TRANSMEM 119..150; /note="Helical"; /evidence="ECO:0000250|UniProtKB:P35523"; TRANSMEM 159..179; /note="Helical"; /evidence="ECO:0000250|UniProtKB:P35523"; TRANSMEM 302..321; /note="Helical"; /evidence="ECO:0000250|UniProtKB:P35523"; TRANSMEM 348..376; /note="Helical"; /evidence="ECO:0000250|UniProtKB:P35523"; TRANSMEM 391..408; /note="Helical"; /evidence="ECO:0000250|UniProtKB:P35523
uniprot
proteins