“Woah! It's like Spotify but for academic articles.”

Instant Access to Thousands of Journals for just $30/month

Get 2 Weeks Free

CBS domains: structure, function, and pathology in human proteins

CBS domains: structure, function, and pathology in human proteins The cystathionine- -synthase (CBS) domain is an evolutionarily conserved protein domain that is present in the proteome of archaebacteria, prokaryotes, and eukaryotes. CBS domains usually come in tandem repeats and are found in cytosolic and membrane proteins performing different functions (metabolic enzymes, kinases, and channels). Crystallographic studies of bacterial CBS domains have shown that two CBS domains form an intramolecular dimeric structure (CBS pair). Several human hereditary diseases (homocystinuria, retinitis pigmentosa, hypertrophic cardiomyopathy, myotonia congenital, etc.) can be caused by mutations in CBS domains of, respectively, cystathionine- -synthase, inosine 5'-monophosphate dehydrogenase, AMP kinase, and chloride channels. Despite their clinical relevance, it remains to be established what the precise function of CBS domains is and how they affect the structural and/or functional properties of an enzyme, kinase, or channel. Depending on the protein in which they occur, CBS domains have been proposed to affect multimerization and sorting of proteins, channel gating, and ligand binding. However, recent experiments revealing that CBS domains can bind adenosine-containing ligands such ATP, AMP, or S -adenosylmethionine have led to the hypothesis that CBS domains function as sensors of intracellular metabolites. chloride channel; cystathionine -synthase; AMP-activated protein kinase Address for reprint requests and other correspondence: J. Eggermont, Laboratory of Physiology, K.U. Leuven, Campus Gasthuisberg O&N, Herestraat 49, B-3000 Leuven, Belgium (e-mail: Jan.Eggermont@med.kuleuven.be ) http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png AJP - Cell Physiology The American Physiological Society

Loading next page...
 
/lp/the-american-physiological-society/cbs-domains-structure-function-and-pathology-in-human-proteins-24yvPK0qsQ

You're reading a free preview. Subscribe to read the entire article.

And millions more from thousands of peer-reviewed journals, for just $30/month

Get 2 Weeks Free

To be the best researcher, you need access to the best research

  • With DeepDyve, you can stop worrying about how much articles cost, or if it's too much hassle to order — it's all at your fingertips. Your research is important and deserves the top content.
  • Read from thousands of the leading scholarly journals from Springer, Elsevier, Nature, IEEE, Wiley-Blackwell and more.
  • All the latest content is available, no embargo periods.

Stop missing out on the latest updates in your field

  • We’ll send you automatic email updates on the keywords and journals you tell us are most important to you.
  • There is a lot of content out there, so we help you sift through it and stay organized.