Search Results - "Christoph O. Randak"

Airway acidification initiates host defense abnormalities in cystic fibrosis mice by Shah, Viral S., Meyerholz, David K., Tang, Xiao Xiao, Reznikov, Leah, Alaiwa, Mahmoud Abou, Ernst, Sarah E., Karp, Philip H., Wohlford-Lenane, Christine L., Heilmann, Kristopher P., Leidinger, Mariah R., Allen, Patrick D., Zabner, Joseph, McCray, Paul B., Ostedgaard, Lynda S., Stoltz, David A., Randak, Christoph O., Welsh, Michael J.

“…Cystic fibrosis (CF) is caused by mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel. In humans…”
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Processing and function of CFTR-ΔF508 are species-dependent by Ostedgaard, Lynda S, Rogers, Christopher S, Dong, Qian, Randak, Christoph O, Vermeer, Daniel W, Rokhlina, Tatiana, Karp, Philip H, Welsh, Michael J

“…Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The most common mutation, a deletion of the phenylalanine at…”
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Increased CFTR expression and function from an optimized lentiviral vector for cystic fibrosis gene therapy by Marquez Loza, Laura I., Cooney, Ashley L., Dong, Qian, Randak, Christoph O., Rivella, Stefano, Sinn, Patrick L., McCray, Paul B.

“…Despite significant advances in cystic fibrosis (CF) treatments, a one-time treatment for this life-shortening disease remains elusive. Stable complementation…”
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CFTR-deficient pigs display peripheral nervous system defects at birth by Reznikov, Leah R., Dong, Qian, Chen, Jeng-Haur, Moninger, Thomas O., Park, Jung Min, Zhang, Yuzhou, Du, Jianyang, Hildebrand, Michael S., Smith, Richard J. H., Randak, Christoph O., Stoltz, David A., Welsh, Michael J.

“…Peripheral nervous system abnormalities, including neuropathy, have been reported in people with cystic fibrosis. These abnormalities have largely been…”
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An elusive adenylate cyclase complicit in cholera is exposed by Randak, Christoph O.

“…The intestinal consequences of cholera enterotoxin are caused by activation of the cystic fibrosis transmembrane conductance regulator (CFTR) anion channel…”
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Curcumin Stimulates Cystic Fibrosis Transmembrane Conductance Regulator Cl– Channel Activity by Berger, Allan L., Randak, Christoph O., Ostedgaard, Lynda S., Karp, Philip H., Vermeer, Daniel W., Welsh, Michael J.

“…Compounds that enhance either the function or biosynthetic processing of the cystic fibrosis transmembrane conductance regulator (CFTR) Cl– channel may be of…”
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A mutation in CFTR modifies the effects of the adenylate kinase inhibitor Ap5A on channel gating by Dong, Qian, Randak, Christoph O, Welsh, Michael J

“…Mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The CFTR anion channel is controlled…”
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ADP Inhibits Function of the ABC Transporter Cystic Fibrosis Transmembrane Conductance Regulator via Its Adenylate Kinase Activity by Randak, Christoph O., Welsh, Michael J.

“…ADP interacts with the nucleotide-binding domains (NBDs) of the cystic fibrosis transmembrane conductance regulator (CFTR) to inhibit its Cl-channel activity…”
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A child with progressive multiple tracheal diverticulae: A variation of the Mounier–Kuhn syndrome by Randak, Christoph O., Weinberger, Miles

“…Mounier–Kuhn syndrome is a rare disorder characterized by tracheobronchomegaly. Most commonly presenting in adults, a broad spectrum of clinical abnormalities…”
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Monocyte derived macrophages from CF pigs exhibit increased inflammatory responses at birth by Paemka, Lily, McCullagh, Brian N, Abou Alaiwa, Mahmoud H, Stoltz, David A, Dong, Qian, Randak, Christoph O, Gray, Robert D, McCray, Paul B

“…Abstract Background We sought to address whether CF macrophages have a primary functional defect as a consequence of CFTR loss and thus contribute to the onset…”
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Mutating the Conserved Q-loop Glutamine 1291 Selectively Disrupts Adenylate Kinase-dependent Channel Gating of the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) and Reduces Channel Function in Primary Human Airway Epithelia by Dong, Qian, Ernst, Sarah E., Ostedgaard, Lynda S., Shah, Viral S., Ver Heul, Amanda R., Welsh, Michael J., Randak, Christoph O.

“…The ATP-binding cassette (ABC) transporter cystic fibrosis transmembrane conductance regulator (CFTR) and two other non-membrane-bound ABC proteins, Rad50 and…”
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Demonstration of Phosphoryl Group Transfer Indicates That the ATP-binding Cassette (ABC) Transporter Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) Exhibits Adenylate Kinase Activity by Randak, Christoph O., Ver Heul, Amanda R., Welsh, Michael J.

“…Cystic fibrosis transmembrane conductance regulator (CFTR) is a membrane-spanning adenosine 5′-triphosphate (ATP)-binding cassette (ABC) transporter. ABC…”
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ATP and AMP Mutually Influence Their Interaction with the ATP-binding Cassette (ABC) Adenylate Kinase Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) at Separate Binding Sites by Randak, Christoph O., Dong, Qian, Ver Heul, Amanda R., Elcock, Adrian H., Welsh, Michael J.

“…Cystic fibrosis transmembrane conductance regulator (CFTR) is an anion channel in the ATP-binding cassette (ABC) transporter protein family. In the presence of…”
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Adenylate Kinase Activity in ABC Transporters by Randak, Christoph O., Welsh, Michael J.

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Role of CFTR's intrinsic adenylate kinase activity in gating of the Cl(-) channel by Randak, Christoph O, Welsh, Michael J

“…The cystic fibrosis transmembrane conductance regulator (CFTR) is a Cl(-)channel in the ATP-binding cassette (ABC) transporter protein family. CFTR features…”
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A Mutation in CFTR Modifies the Effects of the Adenylate Kinase Inhibitor Ap^sub 5^A on Channel Gating by Dong, Qian, Randak, Christoph O, Welsh, Michael J

“…Mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The CFTR anion channel is controlled…”
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A Mutation in CFTR Modifies the Effects of the Adenylate Kinase Inhibitor Ap sub(5A on Channel Gating) by Dong, Qian, Randak, Christoph O, Welsh, Michael J

“…Mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The CFTR anion channel is controlled…”
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A Mutation in CFTR Modifies the Effects of the Adenylate Kinase Inhibitor Ap 5A on Channel Gating by Dong, Qian, Randak, Christoph O., Welsh, Michael J.

“…Mutations in the gene that encodes the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The CFTR anion channel is controlled…”
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Processing and function of CFTR-¿F508 are species-dependent by Ostedgaard, Lynda S, Rogers, Christopher S, Qian Dong, Randak, Christoph O

“…Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The most common mutation, a deletion of the phenylalanine at…”
Get full text

Processing and function of CFTR-DeltaF508 are species-dependent by Ostedgaard, Lynda S, Rogers, Christopher S, Dong, Qian, Randak, Christoph O, Vermeer, Daniel W, Rokhlina, Tatiana, Karp, Philip H, Welsh, Michael J

“…Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) cause cystic fibrosis. The most common mutation, a deletion of the phenylalanine at…”
Get full text