Barbara R Grubb

UNCCH, School of Medicine, Medicine

Scopus Publication Detail

The publication detail shows the title, authors (with indicators showing other profiled authors), information on the publishing organization, abstract and a link to the article in Scopus. This abstract is what is used to create the fingerprint of the publication.

Relationship of a non-cystic fibrosis transmembrane conductance regulator- mediated chloride conductance to organ-level disease in Cftr(-/-) mice

L.L. Clarke; B.R. Grubb; J.R. Yankaskas; C.U. Cotton; A. McKenzie; R.C. Boucher (Profiled Authors: James R Yankaskas; Barbara R Grubb; Richard C Boucher)

Proceedings of the National Academy of Sciences of the United States of America. 1994;91(2):479-483.

Although loss of cystic fibrosis transmembrane conductance regulator (CFTR)-mediated Cl^- channel function is common to all epithelia in cystic fibrosis (CF) patients, the severity of disease varies in different organs. We hypothesized that differences in disease severity in CF relate to the expression of an 'alternative' plasma membrane Cl^- conductance. In CF mice [Cftr(-/-); mice homozygous for Ser-489 to Xaa mutation], which do not express cAMP CFTR-mediated Cl^- secretion, we surveyed organs that exhibit a range of disease severity for a Ca²⁺-mediated apical membrane epithelial Cl^- conductance. This alternative conductance (Cl(a)/^-) was detected in epithelia of organs from CF mice that exhibit a mild disease phenotype (airway, pancreas) but not in epithelia with a severe phenotype (small, large intestine). We conclude that (i) there is an intracellular Ca²⁺-regulated Cl^- conductance that is molecularly distinct from CFTR; and (ii) the level of expression of this alternative Cl^- conductance in the epithelium is an important determinant of the severity of organ-level disease in CF.

PMID: 7507247     PMCID: PMC42972

Scientific Context

This section shows information related to the publication - computed using the fingerprint of the publication - including related publications, related experts with fingerprints representing significant amounts of overlap between their fingerprint and this publication. The red dots indicate whether those experts or terms appear within the publication, thereby showing potential and actual connections.