Search Results - "Kyle, J. W."

Isoform-dependent interaction of voltage-gated sodium channels with protons by Khan, A., Kyle, J. W., Hanck, D. A., Lipkind, G. M., Fozzard, H. A.

“…Protons are potent physiological modifiers of voltage-gated Na + channels, shifting the voltage range of channel gating and reducing current magnitude (p K a…”
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Toxin-Resistant Sodium Channels: Parallel Adaptive Evolution across a Complete Gene Family by Jost, Manda Clair, Hillis, David M., Lu, Ying, Kyle, John W., Fozzard, Harry A., Zakon, Harold H.

“…Approximately 75% of vertebrate proteins belong to protein families encoded by multiple evolutionarily related genes, a pattern that emerged as a result of…”
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The human cation-independent mannose 6-phosphate receptor. Cloning and sequence of the full-length cDNA and expression of functional receptor in COS cells by Oshima, A, Nolan, C M, Kyle, J W, Grubb, J H, Sly, W S

“…We have cloned and sequenced the full-length cDNA for the human cation-independent mannose 6-phosphate receptor from four overlapping clones. The…”
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A murine model of mucopolysaccharidosis VII. Gross and microscopic findings in beta-glucuronidase-deficient mice by Vogler, C, Birkenmeier, EH, Sly, WS, Levy, B, Pegors, C, Kyle, JW, Beamer, WG

“…This report describes the clinical and pathologic alterations found in mice that have a recessively inherited, essentially complete deficiency of the lysosomal…”
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Increased Life Span and Correction of Metabolic Defects in Murine Mucopolysaccharidosis Type VII After Syngeneic Bone Marrow Transplantation by Birkenmeier, Edward H., Barker, Jane E., Vogler, Carole A., Kyle, John W., Sly, William S., Gwynn, Babette, Levy, Beth, Pegors, Catherine

“…The gusfmps/gusmps mouse has no β-glucuronidase activity and develops murine mucopolysaccharidosis type VII (MPS VII). The clinical and pathologic…”
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Two human paramyotonia congenita mutations have opposite effects on lidocaine block of Na+ channels expressed in a mammalian cell line by Z Fan, A L George, Jr, J W Kyle, J C Makielski

“…1. Two mutant human skeletal muscle voltage-gated Na+ channel alpha-subunits (hSkM1), with mutations found in patients with hereditary paramyotonia congenita…”
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Optimization of a mammalian expression system for the measurement of sodium channel gating currents by Sheets, M F, Kyle, J W, Krueger, S, Hanck, D A

“…We describe a new mammalian expression system that optimizes conditions for the measurement of Na channel gating currents (Ig). The small magnitude of Ig…”
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Cloning, Sequencing, and Expression of cDNA for Human β -glucuronidase by Oshima, Akihiro, Kyle, John W., Miller, Raymond D., Hoffmann, Joseph W., Powell, Penelope P., Grubb, Jeffrey H., Sly, William S., Tropak, Michael, Guise, Kevin S., Gravel, Roy A.

“…We report here the cDNA sequence for human placental β -glucuronidase (β -D-glucuronoside glucuronosohydrolase, EC 3.2.1.31) and demonstrate expression of the…”
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Cardiac sodium channels expressed in a peripheral neurotumor-derived cell line, RT4-B8 by Zeng, D, Kyle, J W, Martin, R L, Ambler, K S, Hanck, D A

“…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…”
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Expression of human cation-independent mannose 6-phosphate receptor cDNA in receptor-negative mouse P388D1 cells following gene transfer by Kyle, J W, Nolan, C M, Oshima, A, Sly, W S

“…We recently reported the cDNA cloning, sequence, and expression of the human cation-independent mannose 6-phosphate receptor (hCI-MPR) (Oshima, A., Nolan, C…”
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Correction of Murine Mucopolysaccharidosis VII by a Human β- Glucuronidase Transgene by Kyle, John W., Birkenmeier, Edward H., Gwynn, Babette, Vogler, Carole, Hoppe, Peter C., Hoffmann, Joseph W., Sly, William S.

“…We recently described a murine model for mucopolysaccharidosis VII in mice that have an inherited deficiency of β-glucuronidase (β-D-glucuronoside…”
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The Na Channel Voltage Sensor Associated with Inactivation Is Localized to the External Charged Residues of Domain IV, S4 by Sheets, Michael F., Kyle, John W., Kallen, Roland G., Hanck, Dorothy A.

“…Site-3 toxins have been shown to inhibit a component of gating charge (33% of maximum gating charge, Q max) in native cardiac Na channels that has been…”
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Mucopolysaccharidosis VII: postmortem biochemical and pathological findings in a young adult with beta-glucuronidase deficiency by Vogler, C, Levy, B, Kyle, J W, Sly, W S, Williamson, J, Whyte, M P

“…The postmortem biochemical and pathological findings in the first patient reported with mucopolysaccharidosis VII are described. Clinical, radiographic, and…”
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The saxitoxin/tetrodotoxin binding site on cloned rat brain IIa Na channels is in the transmembrane electric field by Satin, J., Limberis, J.T., Kyle, J.W., Rogart, R.B., Fozzard, H.A.

“…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…”
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The cloned cardiac Na channel α-subunit expressed in Xenopus oocytes show gating and blocking properties of native channels by SATIN, J, KYLE, J. W, CHEN, M, ROGART, R. B, FOZZARD, H. A

“…The neonatal rat cardiac Na channel alpha-subunit directed currents in oocytes show characteristic cardiac relative resistance to tetrodotoxin (TTX) block…”
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Post-repolarization block of cloned sodium channels by saxitoxin: the contribution of pore-region amino acids by Satin, J., Kyle, J.W., Fan, Z., Rogart, R., Fozzard, H.A., Makielski, J.C.

“…Sodium channels expressed in oocytes exhibited isoform differences in phasic block by saxitoxin (STX). Neuronal channels (rat IIa co-expressed with beta 1…”
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Physiological Control of Smooth Muscle-specific Gene Expression through Regulated Nuclear Translocation of Serum Response Factor by Camoretti-Mercado, Blanca, Liu, Hong-W., Halayko, Andrew J., Forsythe, Sean M., Kyle, John W., Li, Bei, Fu, Yiping, McConville, John, Kogut, Paul, Vieira, Joaquim E., Patel, Nina M., Hershenson, Marc B., Fuchs, Elaine, Sinha, Satrajit, Miano, Joseph M., Parmacek, Michael S., Burkhardt, Janis K., Solway, Julian

“…Prolonged serum deprivation induces a structurally and functionally contractile phenotype in about 1/6 of cultured airway myocytes, which exhibit morphological…”
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A Mutant of TTX-Resistant Cardiac Sodium Channels with TTX-Sensitive Properties by Satin, Jonathan, Kyle, John W., Chen, Michael, Bell, Peter, Cribbs, Leanne L., Fozzard, Harry A., Rogart, Richard B.

“…The cardiac sodium channel α subunit (RHI) is less sensitive to tetrodotoxin (TTX) and saxitoxin (STX) and more sensitive to cadmium than brain and skeletal…”
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A Specific Interaction between the Cardiac Sodium Channel and Site-3 Toxin Anthopleurin B by Benzinger, G. Richard, Kyle, John W., Blumenthal, Kenneth M., Hanck, Dorothy A.

“…The polypeptide neurotoxin anthopleurin B (ApB) isolated from the venom of the sea anemone Anthopleura xanthogrammica is one of a family of toxins that bind to…”
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Enzyme replacement therapy for murine mucopolysaccharidosis type VII by Sands, M S, Vogler, C, Kyle, J W, Grubb, J H, Levy, B, Galvin, N, Sly, W S, Birkenmeier, E H

“…Recombinant mouse beta-glucuronidase administered intravenously to newborn mice with mucopolysaccharidosis type VII (MPS VII) is rapidly cleared from the…”
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