Search Results - "WORMAN, H. J"

Nuclear envelope alterations in fibroblasts from patients with muscular dystrophy, cardiomyopathy, and partial lipodystrophy carrying lamin A/C gene mutations by Muchir, A., Medioni, J., Laluc, M., Massart, C., Arimura, T., Kooi, A. J. Van Der, Desguerre, I., Mayer, M., Ferrer, X., Briault, S., Hirano, M., Worman, H. J., Mallet, A., Wehnert, M., Schwartz, K., Bonne, G.

“…Mutations in LMNA, the gene that encodes nuclear lamins A and C, cause up to eight different diseases collectively referred to as “laminopathies.” These…”
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Epitope‐specific anti‐nuclear antibodies are expressed in a mouse model of primary biliary cirrhosis and are cytokine‐dependent by Yang, C‐Y., Leung, P. S. C., Yang, G‐X., Kenny, T. P., Zhang, W., Coppel, R., Norman, G. L., Ansari, A. A., Mackay, I. R., Worman, H. J., Gershwin, M. E.

“…Summary Although the hallmark of primary biliary cirrhosis (PBC) is the presence of anti‐mitochondrial antibodies (AMA), a significant number of patients have…”
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“Atypical p-ANCA” in IBD and hepatobiliary disorders react with a 50-kilodalton nuclear envelope protein of neutrophils and myeloid cell lines by Terjung, Birgit, Spengler, Ulrich, Sauerbruch, Tilman, Worman, Howard J.

“…Background & Aims: Atypical “antineutrophil cytoplasmic antibodies” (ANCA) are present in patients with ulcerative colitis (UC), primary sclerosing cholangitis…”
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Inner nuclear membrane proteins: functions and targeting by Holmer, L, Worman, H J

“…We summarize the properties of integral membrane proteins that reside in the inner nuclear membrane, including lamin B receptor (LBR), lamina-associated…”
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Structural organization of the human gene encoding nuclear lamin A and nuclear lamin C by Lin, F, Worman, H J

“…We have determined the structural organization of the human gene that encodes nuclear lamins A and C, intermediate filament proteins of the nuclear lamina…”
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Decreased and aberrant nuclear lamin expression in gastrointestinal tract neoplasms by Moss, S F, Krivosheyev, V, de Souza, A, Chin, K, Gaetz, H P, Chaudhary, N, Worman, H J, Holt, P R

“…BACKGROUND Altered expression of lamins A/C and B1, constituent proteins of the nuclear lamina, may occur during differentiation and has also been reported in…”
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Differentiation of antineutrophil nuclear antibodies in inflammatory bowel and autoimmune liver diseases from antineutrophil cytoplasmic antibodies (p‐ANCA) using immunofluorescence microscopy by Terjung, B., Worman, H. J., Herzog, V., Sauerbruch, T., Spengler, U.

“…Perinuclear antineutrophil cytoplasmic antibodies (p‐ANCA) directed against cytoplasmic proteins of neutrophils have been studied extensively in patients with…”
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Both lamin A and lamin C mutations cause lamina instability as well as loss of internal nuclear lamin organization by Broers, Jos L.V., Kuijpers, H.J.H., Östlund, C., Worman, H.J., Endert, J., Ramaekers, F.C.S.

“…We have applied the fluorescence loss of intensity after photobleaching (FLIP) technique to study the molecular dynamics and organization of nuclear lamin…”
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Long‐term treatment experience in a subject with Dunnigan‐type familial partial lipodystrophy: efficacy of rosiglitazone by Lüdtke, A., Heck, K., Genschel, J., Mehnert, H., Spuler, S., Worman, H. J., Schmidt, H. H.‐J.

“…Dunnigan‐type familial partial lipodystrophy (FPLD) is caused by mutations in LMNA, the gene that encodes nuclear lamins A and C. FPLD is characterized by…”
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Primary structure analysis and lamin B and DNA binding of human LBR, an integral protein of the nuclear envelope inner membrane by QIAN YE, WORMAN, H. J

“…We have determined the primary structure of human LBR, an integral protein of the nuclear envelope inner membrane, and examined its interactions with lamin B…”
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Abnormal p38  mitogen-activated protein kinase signaling in dilated cardiomyopathy caused by lamin A/C gene mutation by Muchir, A., Wu, W., Choi, J. C., Iwata, S., Morrow, J., Homma, S., Worman, H. J.

“…We previously interrogated the transcriptome in heart tissue from Lmna super(H222P/H222P) mice, a mouse model of cardiomyopathy caused by lamin A/C gene (LMNA)…”
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The Human Lamin B Receptor/Sterol Reductase Multigene Family by Holmer, Lars, Pezhman, Arash, Worman, Howard J.

“…LBR (lamin B receptor) is an integral protein of the inner nuclear membrane encoded by a gene on human chromosome 1q42.1. LBR has a nucleoplasmic,…”
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Inhibition of extracellular signal-regulated kinase signaling to prevent cardiomyopathy caused by mutation in the gene encoding A-type lamins by Muchir, Antoine, Shan, Jian, Bonne, Gisèle, Lehnart, Stephan E., Worman, Howard J.

“…Autosomal Emery–Dreifuss muscular dystrophy and related disorders with dilated cardiomyopathy and variable skeletal muscle involvement are caused by mutations…”
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Signals and Structural Features Involved in Integral Membrane Protein Targeting to the Inner Nuclear Membrane by Soullam, Bruno, Worman, Howard J.

“…We have examined transfected cells by immunofluorescence microscopy to determine the signals and structural features required for the targeting of integral…”
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The inner nuclear membrane by Worman, H J, Courvalin, J C

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A Lamin B Receptor in the Nuclear Envelope by Worman, Howard J., Yuan, Jeffrey, Blobel, Günter, Georgatos, Spyros D.

“…Using a solution binding assay, we show that purified 125I-labeled lamin B binds in a saturable and specific fashion to lamin-depleted avian erythrocyte…”
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Atypical antineutrophil cytoplasmic antibodies with perinuclear fluorescence in chronic inflammatory bowel diseases and hepatobiliary disorders colocalize with nuclear lamina proteins by Terjung, Birgit, Herzog, Volker, Worman, Howard J., Gestmann, Ingo, Bauer, Christoph, Sauerbruch, Tilman, Spengler, Ulrich

“…Antineutrophil cytoplasmic antibodies (ANCA) are frequently associated with chronic inflammatory bowel diseases (IBD) and hepatobiliary disorders. However,…”
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Autoantibodies in Human Chronic Graft-versus-Host Disease after Hematopoietic Cell Transplantation by Quaranta, S., Shulman, H., Ahmed, A., Shoenfeld, Y., Peter, J., McDonald, G.B., Van de Water, J., Coppel, R., Östlund, C., Worman, H.J., Rizzetto, M., Tsuneyama, K., Nakanuma, Y., Ansari, A., Locatelli, F., Paganin, S., Rosina, F., Manns, M., Gershwin, M.E.

“…Primary biliary cirrhosis (PBC) and graft-versus-host disease (GVHD) are thought to have common immunopathologic features and previous studies have reported…”
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The Amino-Terminal Domain of the Lamin B Receptor Is a Nuclear Envelope Targeting Signal by Soullam, Bruno, Worman, Howard J.

“…The lamin B receptor (LBR) is a polytopic protein of the inner nuclear membrane. It is synthesized without a cleavable amino-terminal signal sequence and…”
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Structural Organization of the Human Gene (LMNB1) Encoding Nuclear Lamin B1 by Lin, Feng, Worman, Howard J.

“…We have determined the structural organization of the human gene (LMNB1) that encodes nuclear lamin B1, an intermediate filament protein of the nuclear…”
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