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Richard J. Gregory
Researcher at Genzyme
Publications - 89
Citations - 13312
Richard J. Gregory is an academic researcher from Genzyme. The author has contributed to research in topics: Cystic fibrosis transmembrane conductance regulator & Genetic enhancement. The author has an hindex of 47, co-authored 88 publications receiving 13009 citations. Previous affiliations of Richard J. Gregory include Howard Hughes Medical Institute.
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Journal ArticleDOI
Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis
Seng H. Cheng,Richard J. Gregory,John Marshall,Sucharita Paul,David W. Souza,Gary A. White,Catherine R. O'Riordan,Alan E. Smith +7 more
TL;DR: It is proposed that the mutant versions of CFTR are recognized as abnormal and remain incompletely processed in the endoplasmic reticulum where they are subsequently degraded.
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Demonstration That CFTR Is a Chloride Channel by Alteration of Its Anion Selectivity
Matthew P. Anderson,Richard J. Gregory,Simon Thompson,David W. Souza,Sucharita Paul,Richard C. Mulligan,Alan E. Smith,Michael J. Welsh +7 more
TL;DR: Exression of the cystic fibrosis transmembrane conductance regulator generates adenosine 3',5'-monophosphate (cAMP)-regulated chloride channels, indicating that CFTR is a cAMP-regulated chloride channel and that lysines 95 and 335 determine anion selectivity.
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Adenovirus-mediated gene transfer transiently corrects the chloride transport defect in nasal epithelia of patients with cystic fibrosis
Joseph Zabner,Larry A. Couture,Richard J. Gregory,Scott M. Graham,Alan E. Smith,Michael J. Welsh +5 more
TL;DR: A E1-deficient adenovirus, encoding CFTR, was administered to a defined area of nasal airway epithelium of three individuals with CF, and there was a decrease in the elevated basal transepithelial voltage, and the normal response to a cAMP agonist was restored.
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Expression of cystic fibrosis transmembrane conductance regulator corrects defective chloride channel regulation in cystic fibrosis airway epithelial cells.
Devra P. Rich,Matthew P. Anderson,Richard J. Gregory,Seng H. Cheng,Sucharita Paul,Douglas M. Jefferson,John D. McCann,Katherine W. Klinger,Alan E. Smith,Michael J. Welsh +9 more
TL;DR: Exposure of CFTR to cultured cystic fibrosis airway epithelial cells corrected the Cl− channel defect, demonstrating a causal relationship between mutations in the CFTR gene and defective Cl− transport which is the hallmark of the disease.
Journal ArticleDOI
Phosphorylation of the R domain by cAMP-dependent protein kinase regulates the CFTR chloride channel.
TL;DR: The four phosphorylated events appear to be degenerate: no one site is essential for channel activity, and, at least in the case of serine 660, phosphorylation at one site alone is sufficient for regulation of Cl- channel activity.