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- W2103104037 abstract "Mutations in the ClC-7/Ostm1 ion transporter lead to osteopetrosis and lysosomal storage disease. Its lysosomal localization hitherto precluded detailed functional characterization. Using a mutated ClC-7 that reaches the plasma membrane, we now show that both the aminoterminus and transmembrane span of the Ostm1 β-subunit are required for ClC-7 Cl(-)/H(+)-exchange, whereas the Ostm1 transmembrane domain suffices for its ClC-7-dependent trafficking to lysosomes. ClC-7/Ostm1 currents were strongly outwardly rectifying owing to slow gating of ion exchange, which itself displays an intrinsically almost linear voltage dependence. Reversal potentials of tail currents revealed a 2Cl(-)/1H(+)-exchange stoichiometry. Several disease-causing CLCN7 mutations accelerated gating. Such mutations cluster to the second cytosolic cystathionine-β-synthase domain and potential contact sites at the transmembrane segment. Our work suggests that gating underlies the rectification of all endosomal/lysosomal CLCs and extends the concept of voltage gating beyond channels to ion exchangers." @default.
- W2103104037 created "2016-06-24" @default.
- W2103104037 creator A5001412216 @default.
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- W2103104037 creator A5016899192 @default.
- W2103104037 creator A5035753256 @default.
- W2103104037 creator A5045974509 @default.
- W2103104037 date "2011-04-28" @default.
- W2103104037 modified "2023-10-18" @default.
- W2103104037 title "ClC-7 is a slowly voltage-gated 2Cl<sup>−</sup>/1H<sup>+</sup>-exchanger and requires Ostm1 for transport activity" @default.
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- W2103104037 doi "https://doi.org/10.1038/emboj.2011.137" @default.
- W2103104037 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/3117652" @default.
- W2103104037 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/21527911" @default.
- W2103104037 hasPublicationYear "2011" @default.
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