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Regulatory insertion removal restores maturation, stability and function of DeltaF508 CFTR.
J Mol Biol. 2010 Aug 13;401(2):194-210. doi: 10.1016/j.jmb.2010.06.019. Epub 2010 Jun 16.
J Mol Biol. 2010.
PMID: 20561529
Free PMC article.
We report here that RI deletion enabled F508 CFTR to mature and traffic to the cell surface where it mediated regulated anion efflux and exhibited robust single chloride channel activity. Long-term pulse-chase experiments showed that the mature DeltaRI/DeltaF508 had a T(1/ …
We report here that RI deletion enabled F508 CFTR to mature and traffic to the cell surface where it mediated regulated anion efflux and exh …
Phenotypic abnormalities in long-term surviving cystic fibrosis mice.
Kent G, Oliver M, Foskett JK, Frndova H, Durie P, Forstner J, Forstner GG, Riordan JR, Percy D, Buchwald M.
Kent G, et al. Among authors: riordan jr.
Pediatr Res. 1996 Aug;40(2):233-41. doi: 10.1203/00006450-199608000-00008.
Pediatr Res. 1996.
PMID: 8827771
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Induction of expression of the cystic fibrosis transmembrane conductance regulator.
Breuer W, Kartner N, Riordan JR, Cabantchik ZI.
Breuer W, et al. Among authors: riordan jr.
J Biol Chem. 1992 May 25;267(15):10465-9.
J Biol Chem. 1992.
PMID: 1375221
Free article.
Further activation of Cl- secretion was obtained by additional short term activation by cyclic AMP analogues or cyclic AMP-inducing agents. ...
Further activation of Cl- secretion was obtained by additional short term activation by cyclic AMP analogues or cyclic AMP-inducing a …
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A gene regulating the time dependence of alpha-L-fucosidase concentration is closely linked with the structural gene in man.
Tümmler B, Duthie M, Buchwald M, Riordan JR.
Tümmler B, et al. Among authors: riordan jr.
Hum Genet. 1984;67(4):396-9. doi: 10.1007/BF00291398.
Hum Genet. 1984.
PMID: 6490007
The dependence of the specific activities of human alpha-L-fucosidase and four other lysosomal glycosidases on cell growth was determined in long-term lymphoid cell lines in which the polymorphic forms of alpha-L-fucosidase were identified by isoelectric focusing. ...
The dependence of the specific activities of human alpha-L-fucosidase and four other lysosomal glycosidases on cell growth was determined in …
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Systematic study of the hydrolysis of 4-methylumbelliferylguanidinobenzoate in plasma from patients with cystic fibrosis and controls.
Tümmler B, Seger E, Riordan JR.
Tümmler B, et al. Among authors: riordan jr.
Clin Chim Acta. 1982 Oct 27;125(2):219-32. doi: 10.1016/0009-8981(82)90198-x.
Clin Chim Acta. 1982.
PMID: 6754142
Since the steady-state turnover of substrate is the dominant term of MUGB hydrolysis, the assay in its present form does not meet the requirements of an active-site titration. ...
Since the steady-state turnover of substrate is the dominant term of MUGB hydrolysis, the assay in its present form does not meet the …
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Human fetal liver contains both zinc- and copper-rich forms of metallothionein.
Riordan JR, Richards V.
Riordan JR, et al.
J Biol Chem. 1980 Jun 10;255(11):5380-3.
J Biol Chem. 1980.
PMID: 7372641
Free article.
Metallothionein was purified under anaerobic conditions from livers of human fetuses ranging from 19 weeks gestational age to term. Homogeneous metallothionein obtained in the absence of reducing agents from the soluble fraction of the tissue which contained 24% and 85% of …
Metallothionein was purified under anaerobic conditions from livers of human fetuses ranging from 19 weeks gestational age to term. H …
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