Target intelligence / Profile preview

Chloride channel protein 7 (CLCN7)

Target
CLCN7
Molecular classification
Voltage-gated chloride/proton exchanger, CLC family, Transporter
01

Overview

Chloride channel protein 7 (CLCN7) is a chloride/proton antiporter primarily located in lysosomes and late endosomes. It plays a crucial role in maintaining lysosomal acidification and bone remodeling. Mutations in CLCN7 are associated with autosomal dominant osteopetrosis type II and potentially other lysosomal storage or neurodegenerative diseases. ClC-7 functions as a homodimer, exchanging two chloride ions for one proton. It's essential for osteoclast function by regulating the acidity required for bone resorption.

Other names
ClC-7
02

Biological functions

Lysosomal acidificationEndosomal acidificationBone resorptionChloride transportProton transportpH regulationIon homeostasis
03

Disease associations

Osteopetrosis type IILysosomal storage diseasesNeurodegenerative diseases
04

Safety considerations

Potential for disrupting lysosomal function leading to cellular dysfunctionOff-target effects on other CLC family members

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