epithelial anion transport in health and disease: the role of the slc26 transporters family, no. 273

novartis foundation · wiley

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Reseña del libro

the slc26 family is a recently discovered family of anion transporters that have broad physiological functions since mutations in several members are linked to a variety of diseases. the founding member was slc26a3, mutations in which are associated with congenital cl- diarrhoea. this book describes the slc26 family which comprises at least 10 genes, each of which may encode multiple proteins through splice variants. most members of the family are expressed in the luminal membrane of epithelial cells and they all act as cl-/hco3- exchangers. they are activated by the cystic fibrosis transmembrane conductance regulator, which they activate reciprocally, leading to a model in which the coordinated function of cftr and slc26 transporters mediates epithelial cl- absorption and hco3- secretion. slc26 family members also transport other molecules. slc26a6 acts as a potent oxalate transporter, a critical activity in the kidney. slc26a4 (pendrin) and slc26a5 (prestin) transport sugars, which may explain some of the observed properties of cochlear hair cells in the inner ear.  molecular biological studies suggest that the slc26 transporters interact with other cl- and hco3- transporters, and possibly other regulatory proteins, to form cl- and hco3- transporting complexes in the luminal membrane. this book offers a comprehensive account of the function of this family of anion transporters in transepithelial fluid and electrolyte secretion and contains valuable insight into the diseases that arise when these are peturbed.

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