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O70496

- CLCN7_MOUSE

UniProt

O70496 - CLCN7_MOUSE

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Protein

H(+)/Cl(-) exchange transporter 7

Gene

Clcn7

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 – 2021ChlorideBy similarity
Sitei245 – 2451Mediates proton transfer from the outer aqueous phase to the interior of the protein; involved in linking H(+) and Cl(-) transportBy similarity
Sitei312 – 3121Mediates proton transfer from the protein to the inner aqueous phaseBy similarity
Binding sitei512 – 5121Chloride; via amide nitrogenBy similarity
Binding sitei600 – 6001ChlorideBy similarity

Regions

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

GO - Molecular functioni

  1. antiporter activity Source: UniProtKB-KW
  2. ATP binding Source: UniProtKB-KW
  3. voltage-gated chloride channel activity Source: InterPro

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 1 Publication; Multi-pass membrane protein 1 Publication

Topology

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

GO - Cellular componenti

  1. cytoplasmic vesicle Source: Ensembl
  2. integral component of membrane Source: UniProtKB-KW
  3. lysosomal membrane Source: Ensembl
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 7PRO_0000094453Add
BLAST

Amino acid modifications

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

Keywords - PTMi

Phosphoprotein

Proteomic databases

MaxQBiO70496.
PaxDbiO70496.
PRIDEiO70496.

PTM databases

PhosphoSiteiO70496.

Expressioni

Gene expression databases

BgeeiO70496.
CleanExiMM_CLCN7.
ExpressionAtlasiO70496. baseline and differential.
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 127-629.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini629 – 69365CBS 1PROSITE-ProRule annotationAdd
BLAST
Domaini739 – 79759CBS 2PROSITE-ProRule annotationAdd
BLAST

Motif

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Motifi201 – 2055Selectivity filter part_1By similarity
Motifi243 – 2475Selectivity filter part_2By similarity
Motifi510 – 5145Selectivity filter part_3By similarity

Sequence similaritiesi

Contains 2 CBS domains.PROSITE-ProRule annotation

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

        10         20         30         40         50
MANVSKKVSW SGRDRDDEEG APLLRRTGQP DEETPLLNGA GPGARQSHSA
60 70 80 90 100
LFRIGQMNNV ELDDELLDPE VDPPHTFPKE IPHNEKLLSL KYESLDYDNS
110 120 130 140 150
ENQLFLEEER RINHTAFRTV EIKRWVICAL IGILTGLVAC FIDIVVENLA
160 170 180 190 200
GLKYRVIKDN IDKFTEKGGL SFSLLLWATL NSAFVLVGSV IVAFIEPVAA
210 220 230 240 250
GSGIPQIKCF LNGVKIPHVV RLKTLVIKVS GVILSVVGGL AVGKEGPMIH
260 270 280 290 300
SGSVIAAGIS QGRSTSLKRD FKIFEYFRRD TEKRDFVSAG AAAGVSAAFG
310 320 330 340 350
APVGGVLFSL EEGASFWNQF LTWRIFFASM ISTFTLNFVL SIYHGNMWDL
360 370 380 390 400
SSPGLINFGR FDSEKMAYTI HEIPVFIAMG VVGGILGAVF NALNYWLTMF
410 420 430 440 450
RIRYIHRPCL QVIEAMLVAA VTATVAFVLI YSSRDCQPLQ GSSMSYPLQL
460 470 480 490 500
FCADGEYNSM AAAFFNTPEK SVVSLFHDPP GSYNPMTLGL FTLVYFFLAC
510 520 530 540 550
WTYGLTVSAG VFIPSLLIGA AWGRLFGISL SYLTGAAIWA DPGKYALMGA
560 570 580 590 600
AAQLGGIVRM TLSLTVIMME ATSNVTYGFP IMLVLMTAKI VGDVFIEGLY
610 620 630 640 650
DMHIQLQSVP FLHWEAPVTS HSLTAREVMS TPVTCLRRRE KVGIIVDVLS
660 670 680 690 700
DTASNHNGFP VVEDVGDTQP ARLQGLILRS QLIVLLKHKV FVERSNMGLV
710 720 730 740 750
QRRLRLKDFR DAYPRFPPIQ SIHVSQDERE CTMDLSEFMN PSPYTVPQEA
760 770 780 790 800
SLPRVFKLFR ALGLRHLVVV DNHNQVVGLV TRKDLARYRL GKGGLEELSL

AQT
Length:803
Mass (Da):88,713
Last modified:August 1, 1998 - v1
Checksum:iA7D6DA5791DAA48C
GO

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
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:
EMBLi
GenBanki
DDBJi
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 127-629.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

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

PTM databases

PhosphoSitei O70496.

Proteomic databases

MaxQBi O70496.
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

Bgeei O70496.
CleanExi MM_CLCN7.
ExpressionAtlasi O70496. baseline and differential.
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: November 26, 2014
This is version 129 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).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