Search Results - "Mounkes, Leslie"

Functional Coupling between the Extracellular Matrix and Nuclear Lamina by Wnt Signaling in Progeria by Hernandez, Lidia, Roux, Kyle J., Wong, Esther Sook Miin, Mounkes, Leslie C., Mutalif, Rafidah, Navasankari, Raju, Rai, Bina, Cool, Simon, Jeong, Jae-Wook, Wang, Honghe, Lee, Hyun-Shik, Kozlov, Serguei, Grunert, Martin, Keeble, Thomas, Jones, C. Michael, Meta, Margarita D., Young, Stephen G., Daar, Ira O., Burke, Brian, Perantoni, Alan O., Stewart, Colin L.

“…The segmental premature aging disease Hutchinson-Gilford Progeria (HGPS) is caused by a truncated and farnesylated form of Lamin A. In a mouse model for HGPS,…”
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A progeroid syndrome in mice is caused by defects in A-type lamins by Stewart, Colin L, Mounkes, Leslie C, Kozlov, Serguei, Hernandez, Lidia, Sullivan, Teresa

“…Numerous studies of the underlying causes of ageing have been attempted by examining diseases associated with premature ageing, such as Werner's syndrome and…”
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Expression of an LMNA-N195K variant of A-type lamins results in cardiac conduction defects and death in mice by Mounkes, Leslie C., Kozlov, Serguei V., Rottman, Jeffrey N., Stewart, Colin L.

“…The nuclear lamina is an ∼10 nm thick proteinaceous layer underlying the inner nuclear membrane. The A-type lamins, nuclear intermediate filament proteins…”
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A-Type Lamins Regulate Retinoblastoma Protein Function by Promoting Subnuclear Localization and Preventing Proteasomal Degradation by Johnson, Brett R., Nitta, Ryan T., Frock, Richard L., Mounkes, Leslie, Barbie, David A., Stewart, Colin L., Harlow, Ed, Kennedy, Brian K.

“…The retinoblastoma protein (pRB) is a critical regulator of cell proliferation and differentiation and an important tumor suppressor. In the G1phase of the…”
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Aging and nuclear organization: lamins and progeria by Mounkes, Leslie C, Stewart, Colin L

“…The discoveries of at least eight human diseases arising from mutations in LMNA, which encodes the nuclear A-type lamins, have revealed the nuclear envelope as…”
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Factors influencing the efficiency of cationic liposome-mediated intravenous gene delivery by Liu, Yong, Mounkes, Leslie C, Liggitt, H. Denny, Brown, Carolyn S, Solodin, Igor, Heath, Timothy D, Debs, Robert J

“…We have characterized the relationships between the design of cationic liposomes as a gene transfer vehicle, their resulting biodistribution and processing in…”
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Functional Identification of Distinct Sets of Antitumor Activities Mediated by the FKBP Gene Family by Fong, Sylvia, Mounkes, Leslie, Liu, Yong, Maibaum, Michael, Alonzo, Eric, Desprez, Pierre-Yves, Thor, Ann D., Kashani-Sabet, Mohammed, Debs, Robert J.

“…Assigning biologic function to the many sequenced but still uncharacterized genes remains the greatest obstacle confronting the human genome project…”
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Proteoglycans Mediate Cationic Liposome-DNA Complex-based Gene Delivery in Vitro and in Vivo by Mounkes, Leslie C., Zhong, Wendy, Cipres-Palacin, Guadalupe, Heath, Timothy D., Debs, Robert J.

“…The factors controlling cationic liposome-DNA complex (CLDC)-based gene transfer in cells and in animals are poorly understood. We found that cell surface…”
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Molecular characterization of mutant alleles of the DNA repair/basal transcription factor haywire/ERCC3 in Drosophila by Mounkes, L.C, Fuller, M.T

“…The haywire gene of Drosophila encodes a putative helicase essential for transcription and nucleotide excision repair. A haywire allele encoding a dominant…”
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Evaluation of the role of lipoprotein metabolism genes in systemic cationic liposome-mediated gene transfer in vivo by Mounkes, L C, Zhong, W, de Silva, H V, Handumrongkul, C, Desai, B, Tse, E, Taylor, J M, Debs, R J

“…Germ line gene disruption and gene insertion are often used to study the function of selected genes in vivo. We used selected knockout and transgenic mouse…”
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The laminopathies: nuclear structure meets disease by Mounkes, Leslie, Kozlov, Serguei, Burke, Brian, Stewart, Colin L

“…Most inherited diseases are associated with mutations in a specific gene. Sometimes, mutations in two or more different genes result in diseases with a similar…”
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The DUG gene of Drosophila melanogaster encodes a structural and functional homolog of the S. cerevisiae SUG1 predicted ATPase associated with the 26S proteasome by Mounkes, Leslie C, Fuller, Margaret T

“…The DUG gene of Drosophila encodes a putative ATPase that is a structural and functional homolog of the yeast SUG1 product. When introduced into S. cerevisiae,…”
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Structural Organization and Functions of the Nucleus in Development, Aging, and Disease by Mounkes, Leslie, Stewart, Colin L.

“…In the past four years at least ten different diseases have been shown to be associated with mutations in genes whose products are associated with the nuclear…”
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The A-Type Lamins: Nuclear Structural Proteins as a Focus for Muscular Dystrophy and Cardiovascular Diseases by Mounkes, Leslie C., Burke, Brian, Stewart, Colin L.

“…Mutations in the lamin A (LMNA) gene are associated with the tissue-specific diseases Emery-Dreifuss muscular dystrophy (EDMD), limb girdle muscular dystrophy…”
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The Nuclear Envelope in Muscular Dystrophy and Cardiovascular Diseases by Burke, Brian, Mounkes, Leslie C., Stewart, Colin L.

“…Considerable interest has been focused on the nuclear envelope in recent years following the realization that several human diseases are linked to defects in…”
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A progeroid syndrome in mice is caused by defect in A-type lamins by MOUNKES, Leslie C, KOZLOV, Serguei, HERNANDEZ, Lidia, SULLIVAN, Teresa, STEWART, Colin L

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A Drosophila model for xeroderma pigmentosum and Cockayne's syndrome: haywire encodes the fly homolog of ERCC3, a human excision repair gene by Mounkes, L C, Jones, R S, Liang, B C, Gelbart, W, Fuller, M T

“…The haywire gene of Drosophila encodes a protein with 66% identity to the product of the human ERCC3 gene, associated with xeroderma pigmentosum B (XP-B) and…”
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Mutations in the mouse Lmna gene causing progeria, muscular dystrophy and cardiomyopathy by Kozlov, Serguei, Mounkes, Leslie, Cutler, Dedra, Sullivan, Terry, Hernandez, Lidia, Levy, Nicolas, Rottman, Jeff, Stewart, Colin L

“…At least ten different diseases have been linked to mutations in proteins associated with the nuclear envelope (NE). Eight of these diseases are associated…”
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Isolation and characterization of Haywire, the Drosophila homolog of ERCC3, a human disease gene implicated in both nucleotide excision repair and transcription by Mounkes, Leslie Catherine

“…The haywire gene of Drosophila encodes a protein with 66% identity to the product of the human gene ERCC3, associated with xeroderma pigmentosum B (XP-B) and…”
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In Vivo Transfection of Animals by Intravenous Injection of Cationic Liposome: DNA Complexes by Mounkes, Leslie C., Zhong, Wendy, Young, Jamie K., Liu, Yong, Liggitt, H. Denny, Heath, Timothy D., Demartini, J. C., Debs, Robert J.

“…Abstract Cationic liposome:DNA complex (CLDC)-mediated gene transfer by intravenous injection results in expression of genes of interest in a variety of animal…”
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