Zobrazeno 1 - 10
of 32
pro vyhledávání: '"J W Kyle"'
Publikováno v:
Biophysical Journal. 67(3):1007-1014
The rat brain IIa (BrIIa) Na channel alpha-subunit and the brain beta 1 subunit were coexpressed in Xenopus oocytes, and peak whole-oocyte Na current (INa) was measured at a test potential of -10 mV. Hyperpolarization of the holding potential resulte
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e5940627f53a2dac541df824bcad6a73
Autor:
J W Kyle, Carole Vogler, Babette Gwynn, Edward H. Birkenmeier, Jane E. Barker, Beth Levy, William S. Sly, Catherine Pegors
Publikováno v:
Blood. 78:3081-3092
The gusmps/gusmps mouse has no beta-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic abnormalities are similar to those found in humans with severe MPS VII. Mutant mice are dysmorphic, d
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::75ed8b00ac69a431b10326c522e9477f
https://doi.org/10.1182/blood.v78.11.3081.3081
https://doi.org/10.1182/blood.v78.11.3081.3081
Autor:
E H, Birkenmeier, J E, Barker, C A, Vogler, J W, Kyle, W S, Sly, B, Gwynn, B, Levy, C, Pegors
Publikováno v:
Blood. 78:3081-3092
The gusmps/gusmps mouse has no beta-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic abnormalities are similar to those found in humans with severe MPS VII. Mutant mice are dysmorphic, d
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::a8a705b6d2fe134ca1203f1c1d8c85cf
https://doi.org/10.1182/blood.v78.11.3081.bloodjournal78113081
https://doi.org/10.1182/blood.v78.11.3081.bloodjournal78113081
Autor:
Carole Vogler, William S. Sly, J W Kyle, Babette Gwynn, Joseph W. Hoffmann, Peter C. Hoppe, Edward H. Birkenmeier
Publikováno v:
Proceedings of the National Academy of Sciences. 87:3914-3918
We recently described a murine model for mucopolysaccharidosis VII in mice that have an inherited deficiency of beta-glucuronidase (beta-D-glucuronoside glucuronosohydrolase, EC 3.2.1.31). Affected mice, of genotype gusmps/gusmps, present clinical ma
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2c24583cc4e1ddaf6aa95027f9c9bdfe
https://doi.org/10.1073/pnas.87.10.3914
https://doi.org/10.1073/pnas.87.10.3914
Publikováno v:
The Journal of physiology. 576(Pt 2)
Protons are potent physiological modifiers of voltage-gated Na(+) channels, shifting the voltage range of channel gating and reducing current magnitude (pK(a) approximately 6). We recently showed that proton block of the skeletal muscle isoform (Na(V
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::92a627db3218f94082ab419e880efd08
https://pubmed.ncbi.nlm.nih.gov/16873405
https://pubmed.ncbi.nlm.nih.gov/16873405
Publikováno v:
The American journal of physiology. 270(5 Pt 1)
RT4-B is one of several cell lines derived from a multipotent stem cell line, RT4-AC, which originated from a rat peripheral neurotumor. Based on Northern blot and ribonuclease protection experiments, RT4-B8 cells have been proposed to express rat ca
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::18347b640200cd19fe5b1348bbf72952
https://pubmed.ncbi.nlm.nih.gov/8967455
https://pubmed.ncbi.nlm.nih.gov/8967455
Publikováno v:
Molecular pharmacology. 49(1)
Coexpression of the rat beta 1 subunit with rat brain and skeletal muscle sodium channel alpha subunits in Xenopus oocytes normalizes currents by accelerating sodium current decay kinetics, shifting steady state availability relationships, and accele
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::40870e5a73b89dfc1a392859da6add92
https://pubmed.ncbi.nlm.nih.gov/8569709
https://pubmed.ncbi.nlm.nih.gov/8569709
Autor:
Beth Levy, Carole Vogler, Jeffrey H. Grubb, William S. Sly, Nancy Galvin, J W Kyle, Edward H. Birkenmeier, Mark S. Sands
Publikováno v:
The Journal of clinical investigation. 93(6)
Recombinant mouse beta-glucuronidase administered intravenously to newborn mice with mucopolysaccharidosis type VII (MPS VII) is rapidly cleared from the circulation and localized in many tissues. Here we determine the tissue distribution of injected
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8f411f97aa773c5dc29c7a3863c34762
https://pubmed.ncbi.nlm.nih.gov/8200966
https://pubmed.ncbi.nlm.nih.gov/8200966
Publikováno v:
Biophysical journal. 66(5)
Sodium channels expressed in oocytes exhibited isoform differences in phasic block by saxitoxin (STX). Neuronal channels (rat IIa co-expressed with beta 1 subunit, Br2a + beta 1) had slower kinetics of phasic block for pulse trains than cardiac chann
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::664d9fdbd2252fd22c39c3b17cc476d4
https://pubmed.ncbi.nlm.nih.gov/8061191
https://pubmed.ncbi.nlm.nih.gov/8061191
Publikováno v:
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc. 7(1)
The postmortem biochemical and pathological findings in the first patient reported with mucopolysaccharidosis VII are described. Clinical, radiographic, and biochemical features of this 19-yr-old black man were initially reported in 1973 when, at age
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid________::73fa70d8e6f3b3fec63ebe7994e07d1c
https://pubmed.ncbi.nlm.nih.gov/8159643
https://pubmed.ncbi.nlm.nih.gov/8159643