Format
Sort by

Send to

Choose Destination

Links from PubMed

Items: 1 to 20 of 145

1.

The homozygote VCP(R¹⁵⁵H/R¹⁵⁵H) mouse model exhibits accelerated human VCP-associated disease pathology.

Nalbandian A, Llewellyn KJ, Kitazawa M, Yin HZ, Badadani M, Khanlou N, Edwards R, Nguyen C, Mukherjee J, Mozaffar T, Watts G, Weiss J, Kimonis VE.

PLoS One. 2012;7(9):e46308. doi: 10.1371/journal.pone.0046308. Epub 2012 Sep 28.

2.

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;47(2):260-70. doi: 10.1002/mus.23522. Epub 2012 Nov 21.

3.

The multiple faces of valosin-containing protein-associated diseases: inclusion body myopathy with Paget's disease of bone, frontotemporal dementia, and amyotrophic lateral sclerosis.

Nalbandian A, Donkervoort S, Dec E, Badadani M, Katheria V, Rana P, Nguyen C, Mukherjee J, Caiozzo V, Martin B, Watts GD, Vesa J, Smith C, Kimonis VE.

J Mol Neurosci. 2011 Nov;45(3):522-31. doi: 10.1007/s12031-011-9627-y. Epub 2011 Sep 3. Review.

PMID:
21892620
4.

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 1;19(9):1741-55. doi: 10.1093/hmg/ddq050. Epub 2010 Feb 10.

5.

Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.

Llewellyn KJ, Nalbandian A, Jung KM, Nguyen C, Avanesian A, Mozaffar T, Piomelli D, Kimonis VE.

Hum Mol Genet. 2014 Mar 1;23(5):1333-44. doi: 10.1093/hmg/ddt523. Epub 2013 Oct 24.

6.

Motor neuron involvement in multisystem proteinopathy: implications for ALS.

Benatar M, Wuu J, Fernandez C, Weihl CC, Katzen H, Steele J, Oskarsson B, Taylor JP.

Neurology. 2013 May 14;80(20):1874-80. doi: 10.1212/WNL.0b013e3182929fc3. Epub 2013 May 1.

7.

Slow development of ALS-like spinal cord pathology in mutant valosin-containing protein gene knock-in mice.

Yin HZ, Nalbandian A, Hsu CI, Li S, Llewellyn KJ, Mozaffar T, Kimonis VE, Weiss JH.

Cell Death Dis. 2012 Aug 16;3:e374. doi: 10.1038/cddis.2012.115.

8.

Pathogenic VCP mutations induce mitochondrial uncoupling and reduced ATP levels.

Bartolome F, Wu HC, Burchell VS, Preza E, Wray S, Mahoney CJ, Fox NC, Calvo A, Canosa A, Moglia C, Mandrioli J, Chiò A, Orrell RW, Houlden H, Hardy J, Abramov AY, Plun-Favreau H.

Neuron. 2013 Apr 10;78(1):57-64. doi: 10.1016/j.neuron.2013.02.028. Epub 2013 Mar 14.

9.

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. doi: 10.1083/jcb.200908115.

10.

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 3;7(2):e1001288. doi: 10.1371/journal.pgen.1001288.

11.

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. Epub 2007 Feb 28.

12.

Rapamycin-induced autophagy aggravates pathology and weakness in a mouse model of VCP-associated myopathy.

Ching JK, Weihl CC.

Autophagy. 2013 May;9(5):799-800. doi: 10.4161/auto.23958. Epub 2013 Feb 25.

13.

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;20(2):251-8. doi: 10.1111/j.1468-1331.2012.03831.x. Epub 2012 Aug 20.

14.

mTOR dysfunction contributes to vacuolar pathology and weakness in valosin-containing protein associated inclusion body myopathy.

Ching JK, Elizabeth SV, Ju JS, Lusk C, Pittman SK, Weihl CC.

Hum Mol Genet. 2013 Mar 15;22(6):1167-79. doi: 10.1093/hmg/dds524. Epub 2012 Dec 18.

15.

Targeted excision of VCP R155H mutation by Cre-LoxP technology as a promising therapeutic strategy for valosin-containing protein disease.

Nalbandian A, Llewellyn KJ, Nguyen C, Monuki ES, Kimonis VE.

Hum Gene Ther Methods. 2015 Feb;26(1):13-24. doi: 10.1089/hgtb.2014.096.

16.

Different dynamic movements of wild-type and pathogenic VCPs and their cofactors to damaged mitochondria in a Parkin-mediated mitochondrial quality control system.

Kimura Y, Fukushi J, Hori S, Matsuda N, Okatsu K, Kakiyama Y, Kawawaki J, Kakizuka A, Tanaka K.

Genes Cells. 2013 Dec;18(12):1131-43. doi: 10.1111/gtc.12103. Epub 2013 Nov 12.

17.

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;130(Pt 2):381-93. Epub 2006 Sep 19.

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;19(5):308-15. doi: 10.1016/j.nmd.2009.01.009. Epub 2009 Apr 19. Review.

19.

VCP associated inclusion body myopathy and paget disease of bone knock-in mouse model exhibits tissue pathology typical of human disease.

Badadani M, Nalbandian A, Watts GD, Vesa J, Kitazawa M, Su H, Tanaja J, Dec E, Wallace DC, Mukherjee J, Caiozzo V, Warman M, Kimonis VE.

PLoS One. 2010 Oct 5;5(10). pii: e13183. doi: 10.1371/journal.pone.0013183.

20.

Inclusion body myopathy-associated mutations in p97/VCP impair endoplasmic reticulum-associated degradation.

Weihl CC, Dalal S, Pestronk A, Hanson PI.

Hum Mol Genet. 2006 Jan 15;15(2):189-99. Epub 2005 Dec 1.

Items per page

Supplemental Content

Write to the Help Desk