2j9l: Difference between revisions
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==Overview== | ==Overview== | ||
The ubiquitous CBS domains, which are found as part of cytoplasmic domains, in the ClC family of chloride channels and transporters, have previously, been identified as building blocks for regulatory nucleotide-binding, sites. Here we report the structures of the cytoplasmic domain of the, human transporter ClC-5 in complex with ATP and ADP. The nucleotides bind, to a specific site in the protein. As determined by equilibrium dialysis, the affinities for ATP, ADP and AMP are in the high micromolar range., Point mutations that interfere with nucleotide binding change the, transport behavior of a ClC-5 mutant expressed in Xenopus laevis oocytes., Our results establish the structural and energetic basis for the, interaction of ClC-5 with nucleotides and provide a framework for future, investigations. | The ubiquitous CBS domains, which are found as part of cytoplasmic domains, in the ClC family of chloride channels and transporters, have previously, been identified as building blocks for regulatory nucleotide-binding, sites. Here we report the structures of the cytoplasmic domain of the, human transporter ClC-5 in complex with ATP and ADP. The nucleotides bind, to a specific site in the protein. As determined by equilibrium dialysis, the affinities for ATP, ADP and AMP are in the high micromolar range., Point mutations that interfere with nucleotide binding change the, transport behavior of a ClC-5 mutant expressed in Xenopus laevis oocytes., Our results establish the structural and energetic basis for the, interaction of ClC-5 with nucleotides and provide a framework for future, investigations. | ||
==Disease== | |||
Known diseases associated with this structure: Dent disease OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300008 300008]], Hypophosphatemic rickets OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300008 300008]], Nephrolithiasis, type I OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300008 300008]], Proteinuria, low molecular weight, with hypercalciuric nephrocalcinosis OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=300008 300008]] | |||
==About this Structure== | ==About this Structure== | ||
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[[Category: voltage-gated channel]] | [[Category: voltage-gated channel]] | ||
''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov | ''Page seeded by [http://ispc.weizmann.ac.il/oca OCA ] on Mon Nov 12 22:53:26 2007'' | ||
