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Journal Abstract Search

409 related items for PubMed ID: 20147319

  • 1. Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
    Custer SK, Neumann M, Lu H, Wright AC, Taylor JP.
    Hum Mol Genet; 2010 May 01; 19(9):1741-55. PubMed ID: 20147319
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  • 5. Transgenic expression of inclusion body myopathy associated mutant p97/VCP causes weakness and ubiquitinated protein inclusions in mice.
    Weihl CC, Miller SE, Hanson PI, Pestronk A.
    Hum Mol Genet; 2007 Apr 15; 16(8):919-28. PubMed ID: 17329348
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  • 6. Pathogenic VCP/TER94 alleles are dominant actives and contribute to neurodegeneration by altering cellular ATP level in a Drosophila IBMPFD model.
    Chang YC, Hung WT, Chang YC, Chang HC, Wu CL, Chiang AS, Jackson GR, Sang TK.
    PLoS Genet; 2011 Feb 03; 7(2):e1001288. PubMed ID: 21304887
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  • 8. Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
    Ju JS, Fuentealba RA, Miller SE, Jackson E, Piwnica-Worms D, Baloh RH, Weihl CC.
    J Cell Biol; 2009 Dec 14; 187(6):875-88. PubMed ID: 20008565
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  • 9. Inclusion body myopathy, Paget's disease of the bone and fronto-temporal dementia: a disorder of autophagy.
    Ju JS, Weihl CC.
    Hum Mol Genet; 2010 Apr 15; 19(R1):R38-45. PubMed ID: 20410287
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  • 10. Proteomic analysis of a drosophila IBMPFD model reveals potential pathogenic mechanisms.
    Chan HT, Lee TR, Huang SH, Lee HY, Sang TK, Chan HL, Lyu PC.
    Mol Biosyst; 2012 Jun 15; 8(6):1730-41. PubMed ID: 22481368
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  • 11. Valosin-containing protein and the pathogenesis of frontotemporal dementia associated with inclusion body myopathy.
    Guinto JB, Ritson GP, Taylor JP, Forman MS.
    Acta Neuropathol; 2007 Jul 15; 114(1):55-61. PubMed ID: 17457594
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  • 12. Phenotypic variability in three families with valosin-containing protein mutation.
    Spina S, Van Laar AD, Murrell JR, Hamilton RL, Kofler JK, Epperson F, Farlow MR, Lopez OL, Quinlan J, DeKosky ST, Ghetti B.
    Eur J Neurol; 2013 Feb 15; 20(2):251-8. PubMed ID: 22900631
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  • 13. Pathological consequences of VCP mutations on human striated muscle.
    Hübbers CU, Clemen CS, Kesper K, Böddrich A, Hofmann A, Kämäräinen O, Tolksdorf K, Stumpf M, Reichelt J, Roth U, Krause S, Watts G, Kimonis V, Wattjes MP, Reimann J, Thal DR, Biermann K, Evert BO, Lochmüller H, Wanker EE, Schoser BG, Noegel AA, Schröder R.
    Brain; 2007 Feb 15; 130(Pt 2):381-93. PubMed ID: 16984901
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  • 14. Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
    Rodriguez-Ortiz CJ, Hoshino H, Cheng D, Liu-Yescevitz L, Blurton-Jones M, Wolozin B, LaFerla FM, Kitazawa M.
    Am J Pathol; 2013 Aug 15; 183(2):504-15. PubMed ID: 23747512
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  • 15. The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?
    Clemen CS, Winter L, Strucksberg KH, Berwanger C, Türk M, Kornblum C, Florin A, Aguilar-Pimentel JA, Amarie OV, Becker L, Garrett L, Hans W, Moreth K, Neff F, Pingen L, Rathkolb B, Rácz I, Rozman J, Treise I, Fuchs H, Gailus-Durner V, de Angelis MH, Vorgerd M, Eichinger L, Schröder R.
    Biochem Biophys Res Commun; 2018 Sep 18; 503(4):2770-2777. PubMed ID: 30100055
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  • 16. A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse.
    Nalbandian A, Llewellyn KJ, Badadani M, Yin HZ, Nguyen C, Katheria V, Watts G, Mukherjee J, Vesa J, Caiozzo V, Mozaffar T, Weiss JH, Kimonis VE.
    Muscle Nerve; 2013 Feb 18; 47(2):260-70. PubMed ID: 23169451
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  • 17. IBMPFD Disease-Causing Mutant VCP/p97 Proteins Are Targets of Autophagic-Lysosomal Degradation.
    Bayraktar O, Oral O, Kocaturk NM, Akkoc Y, Eberhart K, Kosar A, Gozuacik D.
    PLoS One; 2016 Feb 18; 11(10):e0164864. PubMed ID: 27768726
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  • 18. Valosin-containing protein disease: inclusion body myopathy with Paget's disease of the bone and fronto-temporal dementia.
    Weihl CC, Pestronk A, Kimonis VE.
    Neuromuscul Disord; 2009 May 18; 19(5):308-15. PubMed ID: 19380227
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  • 19. A unique IBMPFD-related P97/VCP mutation with differential binding pattern and subcellular localization.
    Erzurumlu Y, Kose FA, Gozen O, Gozuacik D, Toth EA, Ballar P.
    Int J Biochem Cell Biol; 2013 Apr 18; 45(4):773-82. PubMed ID: 23333620
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  • 20. VCP/p97 is essential for maturation of ubiquitin-containing autophagosomes and this function is impaired by mutations that cause IBMPFD.
    Tresse E, Salomons FA, Vesa J, Bott LC, Kimonis V, Yao TP, Dantuma NP, Taylor JP.
    Autophagy; 2010 Feb 18; 6(2):217-27. PubMed ID: 20104022
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