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O70496

- CLCN7_MOUSE

UniProt

O70496 - CLCN7_MOUSE

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Protein
H(+)/Cl(-) exchange transporter 7
Gene
Clcn7, Clc7
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

Slowly voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the lysosome lumen.2 Publications

Sites

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Binding sitei202 – 2021Chloride By similarity
Sitei245 – 2451Mediates proton transfer from the outer aqueous phase to the interior of the protein; involved in linking H(+) and Cl(-) transport By similarity
Sitei312 – 3121Mediates proton transfer from the protein to the inner aqueous phase By similarity
Binding sitei512 – 5121Chloride; via amide nitrogen By similarity
Binding sitei600 – 6001Chloride By similarity

Regions

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Nucleotide bindingi656 – 6583ATP By similarity
Nucleotide bindingi781 – 7844ATP By similarity

GO - Molecular functioni

  1. ATP binding Source: UniProtKB-KW
  2. antiporter activity Source: UniProtKB-KW
  3. protein binding Source: IntAct
  4. voltage-gated chloride channel activity Source: InterPro
Complete GO annotation...

GO - Biological processi

  1. response to pH Source: Ensembl
Complete GO annotation...

Keywords - Biological processi

Antiport, Ion transport, Transport

Keywords - Ligandi

ATP-binding, Chloride, Nucleotide-binding

Enzyme and pathway databases

ReactomeiREACT_196640. Stimuli-sensing channels.

Names & Taxonomyi

Protein namesi
Recommended name:
H(+)/Cl(-) exchange transporter 7
Alternative name(s):
Chloride channel 7 alpha subunit
Chloride channel protein 7
Short name:
ClC-7
Gene namesi
Name:Clcn7
Synonyms:Clc7
OrganismiMus musculus (Mouse)
Taxonomic identifieri10090 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
ProteomesiUP000000589: Chromosome 17

Organism-specific databases

MGIiMGI:1347048. Clcn7.

Subcellular locationi

Lysosome membrane; Multi-pass membrane protein 1 Publication

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini1 – 124124Cytoplasmic By similarity
Add
BLAST
Transmembranei125 – 15733Helical; By similarity
Add
BLAST
Transmembranei172 – 19524Helical; By similarity
Add
BLAST
Intramembranei204 – 2118Helical; By similarity
Transmembranei221 – 23919Helical; By similarity
Add
BLAST
Transmembranei245 – 26218Helical; By similarity
Add
BLAST
Intramembranei286 – 29813Helical; By similarity
Add
BLAST
Intramembranei302 – 3109Helical; By similarity
Transmembranei320 – 33920Helical; By similarity
Add
BLAST
Transmembranei373 – 40331Helical; By similarity
Add
BLAST
Transmembranei408 – 43023Helical; By similarity
Add
BLAST
Transmembranei485 – 50521Helical; By similarity
Add
BLAST
Transmembranei510 – 53324Helical; By similarity
Add
BLAST
Intramembranei543 – 55715Helical; By similarity
Add
BLAST
Intramembranei558 – 5603Note=Loop between two helices; By similarity
Intramembranei561 – 57212Helical; By similarity
Add
BLAST
Intramembranei573 – 5764Note=Loop between two helices; By similarity
Transmembranei577 – 59519Helical; By similarity
Add
BLAST
Topological domaini596 – 803208Cytoplasmic By similarity
Add
BLAST

GO - Cellular componenti

  1. cytoplasmic vesicle Source: Ensembl
  2. integral component of membrane Source: UniProtKB-KW
  3. lysosomal membrane Source: UniProtKB-SubCell
Complete GO annotation...

Keywords - Cellular componenti

Lysosome, Membrane

Pathology & Biotechi

Disruption phenotypei

Accumulates undegraded endocyted material in neurons or renal proximal tubular cells. Almost all ClC-7-deficient neurons die.1 Publication

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Chaini1 – 803803H(+)/Cl(-) exchange transporter 7
PRO_0000094453Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei9 – 91Phosphoserine1 Publication

Keywords - PTMi

Phosphoprotein

Proteomic databases

PaxDbiO70496.
PRIDEiO70496.

PTM databases

PhosphoSiteiO70496.

Expressioni

Gene expression databases

ArrayExpressiO70496.
BgeeiO70496.
CleanExiMM_CLCN7.
GenevestigatoriO70496.

Interactioni

Subunit structurei

Chloride channel 7 are heteromers of alpha (CLCN7) and beta (OSTM1) subunits.1 Publication

Binary interactionsi

WithEntry#Exp.IntActNotes
Ostm1Q8BGT07EBI-987482,EBI-987431

Protein-protein interaction databases

BioGridi204933. 1 interaction.
IntActiO70496. 2 interactions.
MINTiMINT-4090953.

Structurei

3D structure databases

ProteinModelPortaliO70496.
SMRiO70496. Positions 127-684, 736-788.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini629 – 69365CBS 1
Add
BLAST
Domaini739 – 79759CBS 2
Add
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi201 – 2055Selectivity filter part_1 By similarity
Motifi243 – 2475Selectivity filter part_2 By similarity
Motifi510 – 5145Selectivity filter part_3 By similarity

Sequence similaritiesi

Contains 2 CBS domains.

Keywords - Domaini

CBS domain, Repeat, Transmembrane, Transmembrane helix

Phylogenomic databases

eggNOGiCOG0038.
HOGENOMiHOG000231081.
HOVERGENiHBG050985.
InParanoidiO70496.
KOiK05016.
OMAiSWSGRDR.
OrthoDBiEOG77Q4W6.
PhylomeDBiO70496.
TreeFamiTF313867.

Family and domain databases

Gene3Di1.10.3080.10. 2 hits.
InterProiIPR000644. CBS_dom.
IPR014743. Cl-channel_core.
IPR001807. Cl-channel_volt-gated.
IPR002249. Cl_channel-7.
[Graphical view]
PfamiPF00571. CBS. 2 hits.
PF00654. Voltage_CLC. 1 hit.
[Graphical view]
PRINTSiPR00762. CLCHANNEL.
PR01118. CLCHANNEL7.
SMARTiSM00116. CBS. 2 hits.
[Graphical view]
SUPFAMiSSF81340. SSF81340. 2 hits.
PROSITEiPS51371. CBS. 2 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

O70496-1 [UniParc]FASTAAdd to Basket

« Hide

MANVSKKVSW SGRDRDDEEG APLLRRTGQP DEETPLLNGA GPGARQSHSA    50
LFRIGQMNNV ELDDELLDPE VDPPHTFPKE IPHNEKLLSL KYESLDYDNS 100
ENQLFLEEER RINHTAFRTV EIKRWVICAL IGILTGLVAC FIDIVVENLA 150
GLKYRVIKDN IDKFTEKGGL SFSLLLWATL NSAFVLVGSV IVAFIEPVAA 200
GSGIPQIKCF LNGVKIPHVV RLKTLVIKVS GVILSVVGGL AVGKEGPMIH 250
SGSVIAAGIS QGRSTSLKRD FKIFEYFRRD TEKRDFVSAG AAAGVSAAFG 300
APVGGVLFSL EEGASFWNQF LTWRIFFASM ISTFTLNFVL SIYHGNMWDL 350
SSPGLINFGR FDSEKMAYTI HEIPVFIAMG VVGGILGAVF NALNYWLTMF 400
RIRYIHRPCL QVIEAMLVAA VTATVAFVLI YSSRDCQPLQ GSSMSYPLQL 450
FCADGEYNSM AAAFFNTPEK SVVSLFHDPP GSYNPMTLGL FTLVYFFLAC 500
WTYGLTVSAG VFIPSLLIGA AWGRLFGISL SYLTGAAIWA DPGKYALMGA 550
AAQLGGIVRM TLSLTVIMME ATSNVTYGFP IMLVLMTAKI VGDVFIEGLY 600
DMHIQLQSVP FLHWEAPVTS HSLTAREVMS TPVTCLRRRE KVGIIVDVLS 650
DTASNHNGFP VVEDVGDTQP ARLQGLILRS QLIVLLKHKV FVERSNMGLV 700
QRRLRLKDFR DAYPRFPPIQ SIHVSQDERE CTMDLSEFMN PSPYTVPQEA 750
SLPRVFKLFR ALGLRHLVVV DNHNQVVGLV TRKDLARYRL GKGGLEELSL 800
AQT 803
Length:803
Mass (Da):88,713
Last modified:August 1, 1998 - v1
Checksum:iA7D6DA5791DAA48C
GO

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF063101
, AF063098, AF063099, AF063100 Genomic DNA. Translation: AAC18832.1.
BC050907 mRNA. Translation: AAH50907.1.
BC053049 mRNA. Translation: AAH53049.1.
BC054799 mRNA. Translation: AAH54799.1.
CCDSiCCDS28509.1.
RefSeqiNP_036060.1. NM_011930.3.
UniGeneiMm.270587.

Genome annotation databases

EnsembliENSMUST00000040729; ENSMUSP00000035964; ENSMUSG00000036636.
GeneIDi26373.
KEGGimmu:26373.
UCSCiuc008azv.1. mouse.

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
AF063101
, AF063098 , AF063099 , AF063100 Genomic DNA. Translation: AAC18832.1 .
BC050907 mRNA. Translation: AAH50907.1 .
BC053049 mRNA. Translation: AAH53049.1 .
BC054799 mRNA. Translation: AAH54799.1 .
CCDSi CCDS28509.1.
RefSeqi NP_036060.1. NM_011930.3.
UniGenei Mm.270587.

3D structure databases

ProteinModelPortali O70496.
SMRi O70496. Positions 127-684, 736-788.
ModBasei Search...

Protein-protein interaction databases

BioGridi 204933. 1 interaction.
IntActi O70496. 2 interactions.
MINTi MINT-4090953.

PTM databases

PhosphoSitei O70496.

Proteomic databases

PaxDbi O70496.
PRIDEi O70496.

Protocols and materials databases

Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENSMUST00000040729 ; ENSMUSP00000035964 ; ENSMUSG00000036636 .
GeneIDi 26373.
KEGGi mmu:26373.
UCSCi uc008azv.1. mouse.

Organism-specific databases

CTDi 1186.
MGIi MGI:1347048. Clcn7.

Phylogenomic databases

eggNOGi COG0038.
HOGENOMi HOG000231081.
HOVERGENi HBG050985.
InParanoidi O70496.
KOi K05016.
OMAi SWSGRDR.
OrthoDBi EOG77Q4W6.
PhylomeDBi O70496.
TreeFami TF313867.

Enzyme and pathway databases

Reactomei REACT_196640. Stimuli-sensing channels.

Miscellaneous databases

NextBioi 304273.
PROi O70496.
SOURCEi Search...

Gene expression databases

ArrayExpressi O70496.
Bgeei O70496.
CleanExi MM_CLCN7.
Genevestigatori O70496.

Family and domain databases

Gene3Di 1.10.3080.10. 2 hits.
InterProi IPR000644. CBS_dom.
IPR014743. Cl-channel_core.
IPR001807. Cl-channel_volt-gated.
IPR002249. Cl_channel-7.
[Graphical view ]
Pfami PF00571. CBS. 2 hits.
PF00654. Voltage_CLC. 1 hit.
[Graphical view ]
PRINTSi PR00762. CLCHANNEL.
PR01118. CLCHANNEL7.
SMARTi SM00116. CBS. 2 hits.
[Graphical view ]
SUPFAMi SSF81340. SSF81340. 2 hits.
PROSITEi PS51371. CBS. 2 hits.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Loss of the ClC-7 chloride channel leads to osteopetrosis in mice and man."
    Kornak U., Kasper D., Boesl M.R., Kaiser E., Schweizer M., Schulz A., Friedrich W., Delling G., Jentsch T.J.
    Cell 104:205-215(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE.
  2. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
    The MGC Project Team
    Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Strain: C57BL/6.
    Tissue: Brain.
  3. "ClC-7 requires Ostm1 as a beta-subunit to support bone resorption and lysosomal function."
    Lange P.F., Wartosch L., Jentsch T.J., Fuhrmann J.C.
    Nature 440:220-223(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, SUBUNIT, INTERACTION WITH OSTM1, SUBCELLULAR LOCATION.
  4. "Lysosomal degradation of endocytosed proteins depends on the chloride transport protein ClC-7."
    Wartosch L., Fuhrmann J.C., Schweizer M., Stauber T., Jentsch T.J.
    FASEB J. 23:4056-4068(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, DISRUPTION PHENOTYPE.
  5. "The phagosomal proteome in interferon-gamma-activated macrophages."
    Trost M., English L., Lemieux S., Courcelles M., Desjardins M., Thibault P.
    Immunity 30:143-154(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-9, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].

Entry informationi

Entry nameiCLCN7_MOUSE
AccessioniPrimary (citable) accession number: O70496
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 21, 2001
Last sequence update: August 1, 1998
Last modified: September 3, 2014
This is version 126 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program

Miscellaneousi

Miscellaneous

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 By similarity.

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. MGD cross-references
    Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
  2. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

Similar proteinsi