Investigating the crosstalk between CLC-1 C-terminal CBS domains and the TM region

Abstract

CLC channels and transporters have large C-terminal domains which contain two cystathionine β-synthetase (CBS) domains. Conformational changes in these domains have been linked to the process of common gating of the CLC dimer. In line with this experimental evidence, mutations in the CBS domains of ClC-1 cause dominantly inherited myotonia. It is not clear how rearrangements that occur in the CBS domains are transmitted to the ion pathway, since CBS domains interact with the TM region at multiple locations.