CLCN7 (chloride voltage-gated channel 7)

2016-10-01  

Identity

HGNC
LOCATION
16p13.3
LOCUSID
ALIAS
CLC-7,CLC7,HOD,OPTA2,OPTB4,PPP1R63
FUSION GENES

Other Information

Locus ID:

NCBI: 1186
MIM: 602727
HGNC: 2025
Ensembl: ENSG00000103249

Variants:

dbSNP: 1186
ClinVar: 1186
TCGA: ENSG00000103249
COSMIC: CLCN7

RNA/Proteins

Gene IDTranscript IDUniprot
ENSG00000103249ENST00000262318H0Y2M6
ENSG00000103249ENST00000382745P51798
ENSG00000103249ENST00000448525P51798
ENSG00000103249ENST00000564568H3BNG8
ENSG00000103249ENST00000569851I3L470

Expression (GTEx)

0
10
20
30
40
50
60

Pathways

PathwaySourceExternal ID
Transmembrane transport of small moleculesREACTOMER-HSA-382551
Ion channel transportREACTOMER-HSA-983712
Stimuli-sensing channelsREACTOMER-HSA-2672351

Protein levels (Protein atlas)

Not detected
Low
Medium
High

References

Pubmed IDYearTitleCitations
388387762024Gain-of-function variants in CLCN7 cause hypopigmentation and lysosomal storage disease.0
388387762024Gain-of-function variants in CLCN7 cause hypopigmentation and lysosomal storage disease.0
381366692023Impaired Autophagic Clearance with a Gain-of-Function Variant of the Lysosomal Cl(-)/H(+) Exchanger ClC-7.3
381366692023Impaired Autophagic Clearance with a Gain-of-Function Variant of the Lysosomal Cl(-)/H(+) Exchanger ClC-7.3
351591752022The Role of the Lysosomal Cl(-)/H(+) Antiporter ClC-7 in Osteopetrosis and Neurodegeneration.7
351591752022The Role of the Lysosomal Cl(-)/H(+) Antiporter ClC-7 in Osteopetrosis and Neurodegeneration.7
331257612021Pathobiologic Mechanisms of Neurodegeneration in Osteopetrosis Derived From Structural and Functional Analysis of 14 ClC-7 Mutants.12
333590072021Transcriptomic and bioinformatic analysis of Clcn7-dependent Autosomal Dominant Osteopetrosis type 2. Preclinical and clinical implications.2
339055942021Efficient generation of osteoclasts from human induced pluripotent stem cells and functional investigations of lethal CLCN7-related osteopetrosis.17
342588312021CLCN7-related neuropathic infantile osteopetrosis in siblings.2
331257612021Pathobiologic Mechanisms of Neurodegeneration in Osteopetrosis Derived From Structural and Functional Analysis of 14 ClC-7 Mutants.12
333590072021Transcriptomic and bioinformatic analysis of Clcn7-dependent Autosomal Dominant Osteopetrosis type 2. Preclinical and clinical implications.2
339055942021Efficient generation of osteoclasts from human induced pluripotent stem cells and functional investigations of lethal CLCN7-related osteopetrosis.17
342588312021CLCN7-related neuropathic infantile osteopetrosis in siblings.2
327492172020Cryo-EM structure of the lysosomal chloride-proton exchanger CLC-7 in complex with OSTM1.32

Citation

Dessen P

CLCN7 (chloride voltage-gated channel 7)

Atlas Genet Cytogenet Oncol Haematol. 2016-10-01

Online version: http://atlasgeneticsoncology.org/gene/56206/clcn7-(chloride-voltage-gated-channel-7)