Search Results - "Linton, Gilda F"

Correction of canine X-linked severe combined immunodeficiency by in vivo retroviral gene therapy by Ravin, Suk See Ting–De, Kennedy, Douglas R., Naumann, Nora, Kennedy, Jeffrey S., Choi, Uimook, Hartnett, Brian J., Linton, Gilda F., Whiting-Theobald, Narda L., Moore, Peter F., Vernau, William, Malech, Harry L., Felsburg, Peter J.

“…X-linked severe combined immunodeficiency (XSCID) is characterized by profound immunodeficiency and early mortality, the only potential cure being…”
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Analysis of Human Phagocyte Flavocytochrome b558 by Mass Spectrometry by Taylor, Ross M., Baniulis, Danas, Burritt, James B., Gripentrog, Jeannie M., Lord, Connie I., Riesselman, Marcia H., Maaty, Walid S., Bothner, Brian P., Angel, Thomas E., Dratz, Edward A., Linton, Gilda F., Malech, Harry L., Jesaitis, Algirdas J.

“…The catalytic core of the phagocyte NADPH oxidase is a heterodimeric integral membrane protein (flavocytochrome b (Cyt b)) that generates superoxide and…”
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WHIM syndrome myelokathexis reproduced in the NOD/SCID mouse xenotransplant model engrafted with healthy human stem cells transduced with C-terminus–truncated CXCR4 by Kawai, Toshinao, Choi, Uimook, Cardwell, Lanise, DeRavin, Suk See, Naumann, Nora, Whiting-Theobald, Narda L., Linton, Gilda F., Moon, Jaehyun, Murphy, Philip M., Malech, Harry L.

“…WHIM(warts, hypogammaglobulinemia, recurrent bacterial infection, and myelokathexis) syndrome is a rare immunodeficiency caused in many cases by autosomal…”
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Gene therapy improves immune function in preadolescents with X-linked severe combined immunodeficiency by Chinen, Javier, Davis, Joie, De Ravin, Suk See, Hay, Beverly N., Hsu, Amy P., Linton, Gilda F., Naumann, Nora, Nomicos, Effie Y.H., Silvin, Christopher, Ulrick, Jean, Whiting-Theobald, Narda L., Malech, Harry L., Puck, Jennifer M.

“…Retroviral gene therapy can restore immunity to infants with X-linked severe combined immunodeficiency (XSCID) caused by mutations in the IL2RG gene encoding…”
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Reversion mutations in patients with leukocyte adhesion deficiency type-1 (LAD-1) by Uzel, Gulbu, Tng, Emilia, Rosenzweig, Sergio D., Hsu, Amy P., Shaw, Jacqueline M., Horwitz, Mitchell E., Linton, Gilda F., Anderson, Stacie M., Kirby, Martha R., Oliveira, Jaõ B., Brown, Margaret R., Fleisher, Thomas A., Law, S. K. Alex, Holland, Steven M.

“…Leukocyte adhesion deficiency type-1 (LAD-1) is an autosomal recessive immunodeficiency caused by mutations in the β2 integrin, CD18, that impair CD11/CD18…”
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Treatment of Chronic Granulomatous Disease with Nonmyeloablative Conditioning and a T-Cell–Depleted Hematopoietic Allograft by Horwitz, Mitchell E, Barrett, A. John, Brown, Margaret R, Carter, Charles S, Childs, Richard, Gallin, John I, Gress, Ronald E, Holland, Steven M, Linton, Gilda F, Miller, Judi A, Leitman, Susan F, Read, Elizabeth J, Schermerhorn, James, Malech, Harry L

“…Chronic granulomatous disease is a rare inherited immunodeficiency disorder characterized by recurrent, often life-threatening bacterial and fungal infections…”
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CXCR4‐Transgene Expression Significantly Improves Marrow Engraftment of Cultured Hematopoietic Stem Cells by Brenner, Sebastian, Whiting‐Theobald, Narda, Kawai, Toshinao, Linton, Gilda F., Rudikoff, Andrew G., Choi, Uimook, Ryser, Martin F., Murphy, Philip M., Sechler, Joan M.G., Malech, Harry L.

“…Hematopoietic stem cells (HSCs) lose marrow reconstitution potential during ex vivo culture. HSC migration to stromal cell–derived factor (SDF)‐1 (CXCL12)…”
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Enhanced function with decreased internalization of carboxy-terminus truncated CXCR4 responsible for WHIM syndrome by Kawai, Toshinao, Choi, Uimook, Whiting-Theobald, Narda L., Linton, Gilda F., Brenner, Sebastian, Sechler, Joan M.G., Murphy, Philip M., Malech, Harry L.

“…WHIM ( warts, hypogammaglobulinemia, recurrent bacterial infection, myelokathexis) syndrome is an autosomal dominant immune deficiency with severe chronic…”
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Diprotin A infusion into nonobese diabetic/severe combined immunodeficiency mice markedly enhances engraftment of human mobilized CD34+ peripheral blood cells by Kawai, Toshinao, Choi, Uimook, Liu, Po-Ching, Whiting-Theobald, Narda L, Linton, Gilda F, Malech, Harry L

“…Hematopoietic stem cell (HSC) graft cell dose impacts significantly on allogeneic transplant. Similarly, HSC gene therapy outcome is affected by loss of…”
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Prolonged Production of NADPH Oxidase-Corrected Granulocytes after Gene Therapy of Chronic Granulomatous Disease by Malech, H L, Maples, P B, Whiting-Theobald, N, Linton, G F, Sekhsaria, S, Vowells, S J, Li, F, Miller, J A, DeCarlo, E, Holland, S M, Leitman, S F, Carter, C S, Butz, R E, Read, E J, Fleisher, T A, Schneiderman, R D, Van Epps, D E, Spratt, S K, Maack, C A, Rokovich, J A, Cohen, L K, Gallin, J I

“…Little is known about the potential for engraftment of autologous hematopoietic stem cells in human adults not subjected to myeloablative conditioning…”
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The Late Dividing Population of γ‐Retroviral Vector Transduced Human Mobilized Peripheral Blood Progenitor Cells Contributes Most to Gene‐Marked Cell Engraftment in Nonobese Diabetic/Severe Combined Immunodeficient Mice by Brenner, Sebastian, Ryser, Martin F., Whiting‐Theobald, Narda L., Gentsch, Marcus, Linton, Gilda F., Malech, Harry L.

“…We used the nonobese diabetic/severe combined immunodeficient (NOD/SCID) mouse model to assess the repopulation potential of subpopulations of mobilized human…”
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Analysis of human phagocyte flavocytochrome b(558) by mass spectrometry by Taylor, Ross M, Baniulis, Danas, Burritt, James B, Gripentrog, Jeannie M, Lord, Connie I, Riesselman, Marcia H, Maaty, Walid S, Bothner, Brian P, Angel, Thomas E, Dratz, Edward A, Linton, Gilda F, Malech, Harry L, Jesaitis, Algirdas J

“…The catalytic core of the phagocyte NADPH oxidase is a heterodimeric integral membrane protein (flavocytochrome b (Cyt b)) that generates superoxide and…”
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Persistent Mycobacterium avium infection following nonmyeloablative allogeneic peripheral blood stem cell transplantation for interferon-γ receptor-1 deficiency by Horwitz, Mitchell E., Uzel, Gulbu, Linton, Gilda F., Miller, Judi A., Brown, Margaret R., Malech, Harry L., Holland, Steven M.

“…Interferon-γ receptor-1 (IFNγR1) deficiency is a rare inherited immunodeficiency. We performed a nonmyeloablative allogeneic stem cell transplantation on a boy…”
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664. WHIM Syndrome Type Myelokathexis In Vivo in the NOD/SCID Mouse Model Following Transplant of Healthy Human Stem Cells Transduced with C-Terminus Truncated CXCR4 by Kawai, Toshinao, Choi, Uimook, Cardwell, Lanise, Deravin, Suk See, Naumann, Nora, Whiting-Theobald, Narda L., Linton, Gilda F., Brenner, Sebastian, Murphy, Philip M., Malech, Harry L.

“…A prominent feature of WHIM syndrome (an immune deficiency) is myelokathexis (a type of premature apoptosis) of mature myeloid cells in bone marrow (BM)…”
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Nuclear-localizing O6-benzylguanine-resistant GFP-MGMT fusion protein as a novel in vivo selection marker by Choi, Uimook, DeRavin, Suk See, Yamashita, Kouhei, Whiting-Theobald, Narda, Linton, Gilda F, Loktionova, Natalia A, Pegg, Anthony E, Malech, Harry L

“…We characterized a novel in vivo selectable fusion protein, green fluorescence protein-O6-benzylguanine (BG)-resistant O6-methylguanine-methyltransferase…”
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Concentrated RD114-pseudotyped MFGS-gp91phox vector achieves high levels of functional correction of the chronic granulomatous disease oxidase defect in NOD/SCID/β2-microglobulin–/– repopulating mobilized human peripheral blood CD34+ cells by Brenner, Sebastian, Whiting-Theobald, Narda L., Linton, Gilda F., Holmes, Kevin L., Anderson-Cohen, Mindy, Kelly, Patrick F., Vanin, Elio F., Pilon, André M., Bodine, David M., Horwitz, Mitchell E., Malech, Harry L.

“…In previous studies amphotropic MFGS-gp91phox (murine onco-retrovirus vector) was used in a clinical trial of X-linked chronic granulomatous disease (X-CGD)…”
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CXCR4‐Transgene Expression Significantly Improves Marrow Engraftment of Cultured Hematopoietic Stem Cells by Brenner, Sebastian, Whiting‐Theobald, Narda, Kawai, Toshinao, Linton, Gilda F., Rudikoff, Andrew G., Choi, Uimook, Ryser, Martin F., Murphy, Philip M., Sechler, Joan M.G., Malech, Harry L.

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Simian immundeficiency virus lentivector corrects human X-linked chronic granulomatous disease in the NOD/SCID mouse xenograft by Naumann, Nora, De Ravin, Suk See, Choi, Uimook, Moayeri, Morvarid, Whiting-Theobald, Narda, Linton, Gilda F., Ikeda, Yasuhiro, Malech, Harry L.

“…X-linked chronic granulomatous disease (X-CGD) is a primary immunodeficiency caused by mutations in the phagocyte NADPH oxidase catalytic subunit gp91 phox …”
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WHIM syndrome myelokathexis reproduced in the NOD/SCID mouse xenotransplant model engrafted with healthy human stem cells transduced with C-terminus-truncated CXCR4. Commentary by DALE, David C, KAWAI, Toshinao, MALECH, Harry L, CHOI, Uimook, CARDWELL, Lanise, SUK SEE DERAVIN, NAUMANN, Nora, WHITING-THEOBALD, Narda L, LINTON, Gilda F, MOON, Jaehyun, MURPHY, Philip M

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Analysis of Human Phagocyte Flavocytochrome b sub(558) by Mass Spectrometry by Taylor, Ross M, Baniulis, Danas, Burritt, James B, Gripentrog, Jeannie M, Lord, Connie I, Riesselman, Marcia H, Maaty, Walid S, Bothner, Brian P, Angel, Thomas E, Dratz, Edward A, Linton, Gilda F, Malech, Harry L, Jesaitis, Algirdas J

“…The catalytic core of the phagocyte NADPH oxidase is a heterodimeric integral membrane protein (flavocytochrome b (Cyt b)) that generates superoxide and…”
Get full text