Protein: H(+)/Cl(-) exchange transporter 6 (Chloride channel protein 6) (ClC-6) (Chloride transport protein 6) (P51797)
Organism: Homo sapiens (Human)
Function: Voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the late endosome lumen. The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons. The presence of conserved gating glutamate residues is typical for family members that function as antiporters.
Subcellular location: Late endosome membrane {ECO:0000269|PubMed:17534424, ECO:0000269|PubMed:33217309}; Multi-pass membrane protein {ECO:0000269|PubMed:17534424}.
Sequence: MAGCRGSLCCCCRWCCCCGERETRTPEELTILGETQEEEDEILPRKDYESLDYDRCINDPYLEVLETMDNKKGRRYEAVKWMVVFAIGVCTGLVGLFVDFFVRLFTQLKFGVVQTSVEECSQKGCLALSLLELLGFNLTFVFLASLLVLIEPVAAGSGIPEVKCYLNGVKVPGIVRLRTLLCKVLGVLFSVAGGLFVEKEGPMIHSGSVVGAGLPQFQSISLRKIQFNFPYFRSDRDKRDFVSAGAAAGVAAAFGAPIGGTLFSLEEGSSFWNQGLTWKVLFCSMSATFTLNFFRSGIQFGSWGSFQLPGLLNFGEFKCSDSDKKCHLWTAMDLGFFVVMGVIGGLLGATFNCLNKRLAKYRMRNVHPKPKLVRVLESLLVSLVTTVVVFVASMVLGECRQMSSSSQIGNDSFQLQVTEDVNSSIKTFFCPNDTYNDMATLFFNPQESAILQLFHQDGTFSPVTLALFFVLYFLLACWTYGISVPSGLFVPSLLCGAAFGRLVANVLKSYIGLGHIYSGTFALIGAAAFLGGVVRMTISLTVILIESTNEITYGLPIMVTLMVAKWTGDFFNKGIYDIHVGLRGVPLLEWETEVEMDKLRASDIMEPNLTYVYPHTRIQSLVSILRTTVHHAFPVVTENRGNEKEFMKGNQLISNNIKFKKSSILTRAGEQRKRSQSMKSYPSSELRNMCDEHIASEEPAEKEDLLQQMLERRYTPYPNLYPDQSPSEDWTMEERFRPLTFHGLILRSQLVTLLVRGVCYSESQSSASQPRLSYAEMAEDYPRYPDIHDLDLTLLNPRMIVDVTPYMNPSPFTVSPNTHVSQVFNLFRTMGLRHLPVVNAVGEIVGIITRHNLTYEFLQARLRQHYQTI
InterPro domains: - IPR001807: Chloride channel (family) [141-589]
- IPR014743: Chloride channel, core (homologous_superfamily) [70-591]
- IPR002248: Chloride channel ClC-6 (family) [2-223]
- IPR046342: CBS domain superfamily (homologous_superfamily) [591-863]
- IPR051280: Voltage-gated chloride channel/antiporter (family) [17-856]
- IPR000644: CBS domain (domain) [601-868]
Interaction partners: - Protein FAM131B
- Methylenetetrahydrofolate reductase
- Osteopetrosis-associated transmembrane protein 1
- Type-1 angiotensin II receptor-associated protein
- UPF0606 protein KIAA1549
- Palmitoyl-protein thioesterase 1
- Serine/threonine-protein kinase B-raf
- Trafficking kinesin-binding protein 1
Gene Ontology annotations:
Molecular Function (MF): GO:0003674 (molecular function), GO:0005488 (binding), GO:0005215 (transporter activity), GO:0022857 (transmembrane transporter activity), GO:0005515 (protein binding), GO:1901702 (salt transmembrane transporter activity), GO:0015075 (monoatomic ion transmembrane transporter activity), GO:0022803 (passive transmembrane transporter activity), GO:0015318 (inorganic molecular entity transmembrane transporter activity), GO:0005216 (monoatomic ion channel activity), GO:0008509 (monoatomic anion transmembrane transporter activity), GO:0015103 (inorganic anion transmembrane transporter activity), GO:0015108 (chloride transmembrane transporter activity), GO:0015267 (channel activity), GO:0022839 (monoatomic ion gated channel activity), GO:0005253 (monoatomic anion channel activity), GO:0022836 (gated channel activity), GO:0005254 (chloride channel activity), GO:0005244 (voltage-gated monoatomic ion channel activity), GO:0008308 (voltage-gated monoatomic anion channel activity), GO:0022832 (voltage-gated channel activity), GO:0005247 (voltage-gated chloride channel activity)
Biological Process (BP): GO:0008150 (biological process), GO:0008152 (metabolic process), GO:0051179 (localization), GO:0048518 (positive regulation of biological process), GO:0050789 (regulation of biological process), GO:0065007 (biological regulation), GO:0009987 (cellular process), GO:0009058 (biosynthetic process), GO:0055085 (transmembrane transport), GO:0019222 (regulation of metabolic process), GO:0006807 (nitrogen compound metabolic process), GO:0051234 (establishment of localization), GO:0071840 (cellular component organization or biogenesis), GO:0071704 (organic substance metabolic process), GO:0044237 (cellular metabolic process), GO:0050794 (regulation of cellular process), GO:0009893 (positive regulation of metabolic process), GO:0044238 (primary metabolic process), GO:0048522 (positive regulation of cellular process), GO:1901576 (organic substance biosynthetic process), GO:1901564 (organonitrogen compound metabolic process), GO:0010604 (positive regulation of macromolecule metabolic process), GO:0046483 (heterocycle metabolic process), GO:0019538 (protein metabolic process), GO:0060255 (regulation of macromolecule metabolic process), GO:0044249 (cellular biosynthetic process), GO:0006725 (cellular aromatic compound metabolic process), GO:0034641 (cellular nitrogen compound metabolic process), GO:0043170 (macromolecule metabolic process), GO:0009891 (positive regulation of biosynthetic process), GO:0006793 (phosphorus metabolic process), GO:1901360 (organic cyclic compound metabolic process), GO:0006139 (nucleobase-containing compound metabolic process), GO:0031325 (positive regulation of cellular metabolic process), GO:0006810 (transport), GO:0009889 (regulation of biosynthetic process), GO:0051171 (regulation of nitrogen compound metabolic process), GO:0051173 (positive regulation of nitrogen compound metabolic process), GO:0031323 (regulation of cellular metabolic process), GO:0016043 (cellular component organization), GO:0080090 (regulation of primary metabolic process), GO:0044260 (cellular macromolecule metabolic process), GO:0009059 (macromolecule biosynthetic process), GO:1901362 (organic cyclic compound biosynthetic process), GO:0010556 (regulation of macromolecule biosynthetic process), GO:0051052 (regulation of DNA metabolic process), GO:0051054 (positive regulation of DNA metabolic process), GO:0019438 (aromatic compound biosynthetic process), GO:0006796 (phosphate-containing compound metabolic process), GO:0006811 (monoatomic ion transport), GO:0010468 (regulation of gene expression), GO:0034654 (nucleobase-containing compound biosynthetic process), GO:0036211 (protein modification process), GO:0010557 (positive regulation of macromolecule biosynthetic process), GO:0045935 (positive regulation of nucleobase-containing compound metabolic process), GO:0043412 (macromolecule modification), GO:0090304 (nucleic acid metabolic process), GO:0018130 (heterocycle biosynthetic process), GO:0031326 (regulation of cellular biosynthetic process), GO:0019219 (regulation of nucleobase-containing compound metabolic process), GO:0006259 (DNA metabolic process), GO:0044271 (cellular nitrogen compound biosynthetic process), GO:0051252 (regulation of RNA metabolic process), GO:0006996 (organelle organization), GO:0031328 (positive regulation of cellular biosynthetic process), GO:0071897 (DNA biosynthetic process), GO:0045911 (positive regulation of DNA recombination), GO:2001141 (regulation of RNA biosynthetic process), GO:0006468 (protein phosphorylation), GO:0006355 (regulation of DNA-templated transcription), GO:0051276 (chromosome organization), GO:0016310 (phosphorylation), GO:2000573 (positive regulation of DNA biosynthetic process), GO:0045005 (DNA-templated DNA replication maintenance of fidelity), GO:0006260 (DNA replication), GO:0006275 (regulation of DNA replication), GO:1902680 (positive regulation of RNA biosynthetic process), GO:0032774 (RNA biosynthetic process), GO:0006357 (regulation of transcription by RNA polymerase II), GO:0006261 (DNA-templated DNA replication), GO:0045893 (positive regulation of DNA-templated transcription), GO:0071103 (DNA conformation change), GO:0090329 (regulation of DNA-templated DNA replication), GO:1903508 (positive regulation of nucleic acid-templated transcription), GO:1903506 (regulation of nucleic acid-templated transcription), GO:0006351 (DNA-templated transcription), GO:0045944 (positive regulation of transcription by RNA polymerase II), GO:0006811 (monoatomic ion transport), GO:0045944 (positive regulation of transcription by RNA polymerase II)
Cellular Component (CC): GO:0005575 (cellular component), GO:0110165 (cellular anatomical entity), GO:0005622 (intracellular anatomical structure), GO:0043226 (organelle), GO:0016020 (membrane), GO:0005737 (cytoplasm), GO:0005773 (vacuole), GO:0031090 (organelle membrane), GO:0043229 (intracellular organelle), GO:0043227 (membrane-bounded organelle), GO:0000323 (lytic vacuole), GO:0043231 (intracellular membrane-bounded organelle), GO:0005774 (vacuolar membrane), GO:0098588 (bounding membrane of organelle), GO:0005764 (lysosome), GO:0098852 (lytic vacuole membrane), GO:0005765 (lysosomal membrane)