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Tytuł :
New Lmna knock-in mice provide a molecular mechanism for the 'segmental aging' in Hutchinson-Gilford progeria syndrome.
Autorzy :
Jung HJ; Molecular Biology Institute.
Tu Y
Yang SH
Tatar A
Nobumori C
Wu D
Young SG
Fong LG
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Źródło :
Human molecular genetics [Hum Mol Genet] 2014 Mar 15; Vol. 23 (6), pp. 1506-15. Date of Electronic Publication: 2013 Nov 07.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural
MeSH Terms :
Cerebellum/*metabolism
Cerebral Cortex/*metabolism
Lamin Type A/*metabolism
MicroRNAs/*metabolism
Nuclear Proteins/*genetics
Progeria/*genetics
Protein Precursors/*genetics
3' Untranslated Regions ; Alleles ; Animals ; Disease Models, Animal ; Female ; Gene Expression Regulation ; Gene Knock-In Techniques ; Lamin Type A/genetics ; Mice ; Mice, Inbred C57BL ; MicroRNAs/genetics ; Mutation ; Progeria/metabolism ; Progeria/pathology
Czasopismo naukowe
Tytuł :
Targeting protein prenylation in progeria.
Autorzy :
Young SG; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA. />Yang SH
Davies BS
Jung HJ
Fong LG
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Źródło :
Science translational medicine [Sci Transl Med] 2013 Feb 06; Vol. 5 (171), pp. 171ps3.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Review
MeSH Terms :
Progeria/*drug therapy
Protein Prenylation/*drug effects
Animals ; Cell Nucleus Shape/drug effects ; Clinical Trials as Topic ; Enzyme Inhibitors/pharmacology ; Enzyme Inhibitors/therapeutic use ; Farnesyltranstransferase/antagonists & inhibitors ; Farnesyltranstransferase/metabolism ; Humans ; Lamins/metabolism ; Progeria/enzymology ; Progeria/genetics ; Progeria/pathology
Czasopismo naukowe
Tytuł :
Absence of progeria-like disease phenotypes in knock-in mice expressing a non-farnesylated version of progerin.
Autorzy :
Yang SH; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA.
Chang SY
Ren S
Wang Y
Andres DA
Spielmann HP
Fong LG
Young SG
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Źródło :
Human molecular genetics [Hum Mol Genet] 2011 Feb 01; Vol. 20 (3), pp. 436-44. Date of Electronic Publication: 2010 Nov 18.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Farnesyltranstransferase/*metabolism
Nuclear Proteins/*genetics
Nuclear Proteins/*metabolism
Progeria/*genetics
Protein Precursors/*genetics
Protein Precursors/*metabolism
Amino Acid Substitution ; Animals ; Enzyme Inhibitors/pharmacology ; Farnesyltranstransferase/antagonists & inhibitors ; Female ; Gene Knock-In Techniques ; Imidazoles/pharmacology ; Lamin Type A/genetics ; Lamin Type A/metabolism ; Male ; Mice ; Mutation ; Nuclear Proteins/chemistry ; Phenotype ; Progeria/metabolism ; Progeria/pathology ; Progeria/physiopathology ; Protein Precursors/chemistry ; Protein Prenylation
Czasopismo naukowe
Tytuł :
Investigating the purpose of prelamin A processing.
Autorzy :
Davies BS; Department of Medicine, University of California, Los Angeles, USA.
Coffinier C
Yang SH
Barnes RH 2nd
Jung HJ
Young SG
Fong LG
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Źródło :
Nucleus (Austin, Tex.) [Nucleus] 2011 Jan-Feb; Vol. 2 (1), pp. 4-9.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Lamins/*metabolism
Nuclear Proteins/*metabolism
Progeria/*metabolism
Protein Precursors/*metabolism
Animals ; Lamin Type A ; Lamins/deficiency ; Lamins/genetics ; Mice ; Progeria/pathology ; Protein Prenylation ; Protein Processing, Post-Translational
Czasopismo naukowe
Tytuł :
Assessing the efficacy of protein farnesyltransferase inhibitors in mouse models of progeria.
Autorzy :
Yang SH; Department of Medicine, University of California, Los Angeles, Los Angeles, CA.
Chang SY
Andres DA
Spielmann HP
Young SG
Fong LG
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Źródło :
Journal of lipid research [J Lipid Res] 2010 Feb; Vol. 51 (2), pp. 400-5. Date of Electronic Publication: 2009 Oct 26.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Enzyme Inhibitors/*pharmacology
Farnesyltranstransferase/*antagonists & inhibitors
Progeria/*drug therapy
Progeria/*enzymology
Adipose Tissue/drug effects ; Adipose Tissue/metabolism ; Animals ; Body Weight/drug effects ; Disease Models, Animal ; Enzyme Inhibitors/therapeutic use ; Farnesyltranstransferase/metabolism ; Female ; Fibroblasts/drug effects ; Fibroblasts/metabolism ; Imidazoles/pharmacology ; Imidazoles/therapeutic use ; Male ; Mice ; Phenotype ; Prenylation/drug effects ; Progeria/metabolism ; Progeria/pathology ; Survival Analysis ; Time Factors
Czasopismo naukowe
Tytuł :
Activating the synthesis of progerin, the mutant prelamin A in Hutchinson-Gilford progeria syndrome, with antisense oligonucleotides.
Autorzy :
Fong LG; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA. />Vickers TA
Farber EA
Choi C
Yun UJ
Hu Y
Yang SH
Coffinier C
Lee R
Yin L
Davies BS
Andres DA
Spielmann HP
Bennett CF
Young SG
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Źródło :
Human molecular genetics [Hum Mol Genet] 2009 Jul 01; Vol. 18 (13), pp. 2462-71. Date of Electronic Publication: 2009 Apr 17.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Mutation*
Nuclear Proteins/*biosynthesis
Nuclear Proteins/*genetics
Oligonucleotides, Antisense/*therapeutic use
Progeria/*genetics
Progeria/*therapy
Protein Precursors/*biosynthesis
Protein Precursors/*genetics
Alternative Splicing ; Base Sequence ; Cell Line ; Cells, Cultured ; Exons ; Fibroblasts/metabolism ; Genetic Therapy ; Humans ; Lamin Type A ; Molecular Sequence Data ; Oligonucleotides, Antisense/genetics ; Oligonucleotides, Antisense/metabolism ; Progeria/metabolism
Czasopismo naukowe
Tytuł :
Increasing the length of progerin's isoprenyl anchor does not worsen bone disease or survival in mice with Hutchinson-Gilford progeria syndrome.
Autorzy :
Davies BS; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA.
Yang SH
Farber E
Lee R
Buck SB
Andres DA
Spielmann HP
Agnew BJ
Tamanoi F
Fong LG
Young SG
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Źródło :
Journal of lipid research [J Lipid Res] 2009 Jan; Vol. 50 (1), pp. 126-34. Date of Electronic Publication: 2008 Aug 29.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Bone Diseases/*pathology
Nuclear Proteins/*physiology
Progeria/*genetics
Protein Precursors/*physiology
Animals ; Base Sequence ; Bone Diseases/metabolism ; Genotype ; Lamin Type A/metabolism ; Lipids/chemistry ; Mice ; Mice, Transgenic ; Models, Biological ; Models, Genetic ; Molecular Sequence Data ; Nuclear Proteins/genetics ; Nuclear Proteins/metabolism ; Progeria/metabolism ; Protein Precursors/genetics ; Protein Precursors/metabolism ; Syndrome ; Tissue Distribution
Czasopismo naukowe
Tytuł :
The posttranslational processing of prelamin A and disease.
Autorzy :
Davies BS; Department of Medicine, University of California, Los Angeles, California 90095, USA. />Fong LG
Yang SH
Coffinier C
Young SG
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Źródło :
Annual review of genomics and human genetics [Annu Rev Genomics Hum Genet] 2009; Vol. 10, pp. 153-74.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Protein Processing, Post-Translational*
Nuclear Proteins/*metabolism
Progeria/*metabolism
Protein Precursors/*metabolism
Animals ; Disease Models, Animal ; Enzyme Inhibitors/therapeutic use ; Farnesyltranstransferase/antagonists & inhibitors ; Humans ; Infant, Newborn ; Lamin Type A ; Nuclear Proteins/genetics ; Progeria/drug therapy ; Progeria/genetics ; Protein Precursors/genetics
Czasopismo naukowe
Tytuł :
Progerin elicits disease phenotypes of progeria in mice whether or not it is farnesylated.
Autorzy :
Yang SH; Department of Medicine, UCLA David Geffen School of Medicine, Los Angeles, California, USA.
Andres DA
Spielmann HP
Young SG
Fong LG
Pokaż więcej
Źródło :
The Journal of clinical investigation [J Clin Invest] 2008 Oct; Vol. 118 (10), pp. 3291-300.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Prenylation*
Nuclear Proteins/*metabolism
Progeria/*physiopathology
Protein Precursors/*metabolism
Animals ; Blotting, Western ; Body Weight ; Bone and Bones/abnormalities ; Cell Nucleus Shape ; Disease Models, Animal ; Female ; Fibroblasts/cytology ; Fibroblasts/metabolism ; Gene Expression Regulation ; Lamin Type A ; Liver/metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Transgenic ; Nuclear Proteins/genetics ; Phenotype ; Point Mutation ; Progeria/mortality ; Protein Precursors/genetics ; Survival Analysis
Czasopismo naukowe
Tytuł :
Eliminating the synthesis of mature lamin A reduces disease phenotypes in mice carrying a Hutchinson-Gilford progeria syndrome allele.
Autorzy :
Yang SH; Department of Medicine, David Geffen School of Medicine, UCLA, Los Angeles, California 90095, USA.
Qiao X
Farber E
Chang SY
Fong LG
Young SG
Pokaż więcej
Źródło :
The Journal of biological chemistry [J Biol Chem] 2008 Mar 14; Vol. 283 (11), pp. 7094-9. Date of Electronic Publication: 2008 Jan 04.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Gene Expression Regulation*
Lamin Type A/*biosynthesis
Lamin Type A/*genetics
Progeria/*genetics
Alleles ; Animals ; Cell Nucleus/metabolism ; Disease Models, Animal ; Fibroblasts/metabolism ; Fractures, Bone/metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Nuclear Proteins/genetics ; Phenotype ; Protein Precursors/genetics
Czasopismo naukowe
Tytuł :
Treatment with a farnesyltransferase inhibitor improves survival in mice with a Hutchinson-Gilford progeria syndrome mutation.
Autorzy :
Yang SH; Department of Medicine/Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles, Los Angeles, CA, USA.
Qiao X
Fong LG
Young SG
Pokaż więcej
Źródło :
Biochimica et biophysica acta [Biochim Biophys Acta] 2008 Jan-Feb; Vol. 1781 (1-2), pp. 36-9. Date of Electronic Publication: 2007 Nov 26.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Farnesyltranstransferase/*antagonists & inhibitors
Progeria/*drug therapy
Progeria/*genetics
Animals ; Farnesyltranstransferase/metabolism ; Female ; Lamin Type A/genetics ; Lamin Type A/metabolism ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Transgenic ; Mutation ; Phenotype ; Progeria/enzymology ; Survival Rate
Czasopismo naukowe
Tytuł :
Prelamin A farnesylation and progeroid syndromes.
Autorzy :
Young SG; Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, California 90095, USA. />Meta M
Yang SH
Fong LG
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Źródło :
The Journal of biological chemistry [J Biol Chem] 2006 Dec 29; Vol. 281 (52), pp. 39741-5. Date of Electronic Publication: 2006 Nov 07.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Nuclear Proteins/*metabolism
Progeria/*metabolism
Protein Precursors/*metabolism
Animals ; Humans ; Lamin Type A ; Nuclear Proteins/chemistry ; Nuclear Proteins/genetics ; Nuclear Proteins/physiology ; Progeria/etiology ; Progeria/genetics ; Progeria/pathology ; Protein Precursors/chemistry ; Protein Precursors/genetics ; Protein Precursors/physiology ; Protein Prenylation/genetics ; Syndrome
Czasopismo naukowe
Tytuł :
Protein farnesyltransferase inhibitors and progeria.
Autorzy :
Meta M; Department of Medicine, Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA.
Yang SH
Bergo MO
Fong LG
Young SG
Pokaż więcej
Źródło :
Trends in molecular medicine [Trends Mol Med] 2006 Oct; Vol. 12 (10), pp. 480-7. Date of Electronic Publication: 2006 Aug 30.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Review
MeSH Terms :
Alkyl and Aryl Transferases/*antagonists & inhibitors
Enzyme Inhibitors/*therapeutic use
Progeria/*drug therapy
Alkyl and Aryl Transferases/genetics ; Animals ; Disease Models, Animal ; Enzyme Inhibitors/pharmacology ; Humans ; Lamin Type A/biosynthesis ; Membrane Proteins/metabolism ; Metalloendopeptidases/metabolism ; Mice ; Progeria/genetics ; Progeria/metabolism ; Protein Processing, Post-Translational
Czasopismo naukowe
Tytuł :
A farnesyltransferase inhibitor improves disease phenotypes in mice with a Hutchinson-Gilford progeria syndrome mutation.
Autorzy :
Yang SH; Department of Medicine, Division of Cardiology, David Geffen School of Medicine, UCLA, Los Angeles, CA 90095, USA, and Department of Medicine, Wallenberg Laboratory, Sahlgrenska University Hospital, Göteborg, Sweden.
Meta M
Qiao X
Frost D
Bauch J
Coffinier C
Majumdar S
Bergo MO
Young SG
Fong LG
Pokaż więcej
Źródło :
The Journal of clinical investigation [J Clin Invest] 2006 Aug; Vol. 116 (8), pp. 2115-21.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Enzyme Inhibitors/*therapeutic use
Farnesyltranstransferase/*antagonists & inhibitors
Progeria/*genetics
Animals ; Bone Diseases/diagnostic imaging ; Bone and Bones/diagnostic imaging ; Disease Models, Animal ; Mice ; Mutation ; Progeria/diagnostic imaging ; Progeria/drug therapy ; Radiography
Czasopismo naukowe
Tytuł :
A protein farnesyltransferase inhibitor ameliorates disease in a mouse model of progeria.
Autorzy :
Fong LG; Department of Medicine/Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA. />Frost D
Meta M
Qiao X
Yang SH
Coffinier C
Young SG
Pokaż więcej
Źródło :
Science (New York, N.Y.) [Science] 2006 Mar 17; Vol. 311 (5767), pp. 1621-3. Date of Electronic Publication: 2006 Feb 16.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't
MeSH Terms :
Enzyme Inhibitors/*therapeutic use
Farnesyltranstransferase/*antagonists & inhibitors
Imidazoles/*therapeutic use
Progeria/*drug therapy
Animals ; Body Weight/drug effects ; Disease Models, Animal ; Enzyme Inhibitors/pharmacology ; Female ; Hand Strength ; Imidazoles/pharmacology ; Lamin Type A ; Male ; Membrane Proteins/deficiency ; Membrane Proteins/genetics ; Metalloendopeptidases/deficiency ; Metalloendopeptidases/genetics ; Mice ; Nuclear Proteins/metabolism ; Progeria/physiopathology ; Protein Precursors/metabolism ; Protein Prenylation/drug effects ; Rib Fractures/prevention & control ; Survival Rate
Czasopismo naukowe
Tytuł :
Blocking protein farnesyltransferase improves nuclear shape in fibroblasts from humans with progeroid syndromes.
Autorzy :
Toth JI; Department of Medicine and Division of Cardiology, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA.
Yang SH
Qiao X
Beigneux AP
Gelb MH
Moulson CL
Miner JH
Young SG
Fong LG
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Źródło :
Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2005 Sep 06; Vol. 102 (36), pp. 12873-8. Date of Electronic Publication: 2005 Aug 29.
Typ publikacji :
Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Alkyl and Aryl Transferases/*antagonists & inhibitors
Alkyl and Aryl Transferases/*metabolism
Cell Nucleus/*enzymology
Fibroblasts/*cytology
Progeria/*enzymology
Progeria/*pathology
Animals ; Cell Nucleus/drug effects ; Cells, Cultured ; Enzyme Inhibitors/pharmacology ; Farnesyltranstransferase ; Humans ; Lamin Type A ; Membrane Proteins/deficiency ; Membrane Proteins/genetics ; Membrane Proteins/metabolism ; Metalloendopeptidases/deficiency ; Metalloendopeptidases/genetics ; Metalloendopeptidases/metabolism ; Mice ; Nuclear Proteins/metabolism ; Protein Precursors/metabolism
Czasopismo naukowe
Tytuł :
Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.
Autorzy :
Yang SH; Division of Cardiology, Department of Medicine, David Geffen School of Medicine, University of California, Los Angeles, CA 90095, USA.
Bergo MO
Toth JI
Qiao X
Hu Y
Sandoval S
Meta M
Bendale P
Gelb MH
Young SG
Fong LG
Pokaż więcej
Źródło :
Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2005 Jul 19; Vol. 102 (29), pp. 10291-6. Date of Electronic Publication: 2005 Jul 12.
Typ publikacji :
Comparative Study; Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Alkyl and Aryl Transferases/*antagonists & inhibitors
Cell Nucleus/*pathology
Nuclear Proteins/*genetics
Progeria/*metabolism
Protein Precursors/*genetics
Quinolines/*pharmacology
Animals ; Blotting, Southern ; Cell Nucleus/drug effects ; DNA Primers ; Farnesyltranstransferase ; Fibroblasts/drug effects ; Fibroblasts/metabolism ; Genetic Vectors ; Lamin Type A ; Mice ; Mice, Transgenic ; Microscopy, Fluorescence ; Progeria/genetics ; Progeria/pathology ; Sequence Analysis, DNA
Czasopismo naukowe
Tytuł :
Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice.
Autorzy :
Fong LG; Department of Medicine, University of California, Los Angeles, CA 90095, USA. />Ng JK
Meta M
Coté N
Yang SH
Stewart CL
Sullivan T
Burghardt A
Majumdar S
Reue K
Bergo MO
Young SG
Pokaż więcej
Źródło :
Proceedings of the National Academy of Sciences of the United States of America [Proc Natl Acad Sci U S A] 2004 Dec 28; Vol. 101 (52), pp. 18111-6. Date of Electronic Publication: 2004 Dec 17.
Typ publikacji :
Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S.
MeSH Terms :
Heterozygote*
Lamins/*genetics
Lipoproteins/*genetics
Membrane Proteins/*genetics
Metalloendopeptidases/*genetics
Metalloproteases/*genetics
Progeria/*genetics
Alleles ; Animals ; Blotting, Western ; Cell Nucleus/metabolism ; Cell Proliferation ; Cells, Cultured ; Coloring Agents/pharmacology ; Female ; Fibroblasts/metabolism ; Fluorescent Dyes/pharmacology ; Humans ; Lamin Type A ; Lasers ; Mice ; Mice, Knockout ; Mice, Transgenic ; Microscopy, Fluorescence ; Muscles/pathology ; Nuclear Proteins/metabolism ; Organic Chemicals ; Phenotype ; Progeria/pathology ; Protein Precursors/metabolism ; Skull/abnormalities ; Skull/pathology ; Time Factors ; Tomography, X-Ray Computed
Czasopismo naukowe
Tytuł :
Heterozygosity for Lmna Deficiency Eliminates the Progeria-Like Phenotypes in Zmpste24-Deficient Mice
Autorzy :
Loren G. Fong
Jennifer K. Ng
Margarita Meta
Nathan Coté
Shao H. Yang
Colin L. Stewart
Terry Sullivan
Andrew Burghardt
Sharmila Majumdar
Karen Reue
Martin O. Bergo
Stephen G. Young
Francis S. Collins
Pokaż więcej
Źródło :
Proceedings of the National Academy of Sciences of the United States of America. 101(52):18111-18116
Dostęp URL :
https://www.jstor.org/stable/3374202
Czasopismo naukowe

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