Article
Genotypic analysis of respiratory mucous sulfation defects in cystic fibrosis.
The Journal of clinical investigation - 1 Dec 1995
Zhang Y, Doranz B, Yankaskas J R, Engelhardt J F
Abstract excerpt
Intracellular dysfunction of the cystic fibrosis transmembrane conductance regulator (CFTR) has been proposed to alter endosomal acidification. The most widely studied consequence of this defect has been alterations in the biochemical properties of cystic fibrosis (CF) respiratory mucus glycoproteins. However, studies confirming the existence of mucous processing defects in CF have been hindered by the lack of in...
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