Reaction: CLCN7:OSTM1 exchanges Cl- for H+
- in pathway: Stimuli-sensing channels
Chloride channel 7 comprises H+/Cl- exchange transporter 7 (CLCN7) and osteopetrosis-associated transmembrane protein 1 (OSTM1) (Leisle et al. 2011). This complex localises to the lysosomal membrane where it mediates the exchange of Cl- and H+ ions, perhaps playing a role in the acidification of the lysosome (Graves et al. 2008).
Defects in CLCN7 cause osteopetrosis autosomal recessive types 2 and 4 (OPTB2, MIM:166600 and OPTB4, MIM:611490) (Frattini et al. 2003, Pangrazio et al. 2010). Defects in OSTM1 cause osteopetrosis autosomal recessive type 5 (OPTB5, MIM:259720) (Pangrazio et al. 2006).
Defects in CLCN7 cause osteopetrosis autosomal recessive types 2 and 4 (OPTB2, MIM:166600 and OPTB4, MIM:611490) (Frattini et al. 2003, Pangrazio et al. 2010). Defects in OSTM1 cause osteopetrosis autosomal recessive type 5 (OPTB5, MIM:259720) (Pangrazio et al. 2006).
Reaction - small molecule participants:
H+ [lysosomal lumen]
Cl- [cytosol]
Cl- [lysosomal lumen]
H+ [cytosol]
Reactome.org reaction link: R-HSA-2730959
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Reaction input - small molecules:
chloride
hydron
Reaction output - small molecules:
hydron
chloride
Reactome.org link: R-HSA-2730959