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1.
Ceramide contributes to pathogenesis and may be targeted for therapy in VCP inclusion body myopathy.
Hum Mol Genet
; 29(24): 3945-3953, 2021 02 25.
Article
in English
| MEDLINE | ID: mdl-33410456
2.
Lipid-enriched diet rescues lethality and slows down progression in a murine model of VCP-associated disease.
Hum Mol Genet
; 23(5): 1333-44, 2014 Mar 01.
Article
in English
| MEDLINE | ID: mdl-24158850
3.
Administration of CoQ10 analogue ameliorates dysfunction of the mitochondrial respiratory chain in a mouse model of Angelman syndrome.
Neurobiol Dis
; 76: 77-86, 2015 Apr.
Article
in English
| MEDLINE | ID: mdl-25684537
4.
A progressive translational mouse model of human valosin-containing protein disease: the VCP(R155H/+) mouse.
Muscle Nerve
; 47(2): 260-70, 2013 Feb.
Article
in English
| MEDLINE | ID: mdl-23169451
5.
A-form DNA structure is a determinant of transcript levels from the Xenopus gata2 promoter in embryos.
Biochim Biophys Acta
; 1789(11-12): 675-80, 2009.
Article
in English
| MEDLINE | ID: mdl-19665599
6.
Activation of the NLRP3 Inflammasome Is Associated with Valosin-Containing Protein Myopathy.
Inflammation
; 40(1): 21-41, 2017 Feb.
Article
in English
| MEDLINE | ID: mdl-27730320
7.
Myogenic differentiation of VCP disease-induced pluripotent stem cells: A novel platform for drug discovery.
PLoS One
; 12(6): e0176919, 2017.
Article
in English
| MEDLINE | ID: mdl-28575052
8.
A Fine Balance of Dietary Lipids Improves Pathology of a Murine Model of VCP-Associated Multisystem Proteinopathy.
PLoS One
; 10(7): e0131995, 2015.
Article
in English
| MEDLINE | ID: mdl-26134519
9.
Rapamycin and chloroquine: the in vitro and in vivo effects of autophagy-modifying drugs show promising results in valosin containing protein multisystem proteinopathy.
PLoS One
; 10(4): e0122888, 2015.
Article
in English
| MEDLINE | ID: mdl-25884947
10.
Targeted excision of VCP R155H mutation by Cre-LoxP technology as a promising therapeutic strategy for valosin-containing protein disease.
Hum Gene Ther Methods
; 26(1): 13-24, 2015 Feb.
Article
in English
| MEDLINE | ID: mdl-25545721
11.
Global gene expression profiling in R155H knock-in murine model of VCP disease.
Clin Transl Sci
; 8(1): 8-16, 2015 Feb.
Article
in English
| MEDLINE | ID: mdl-25388089
12.
In vitro studies in VCP-associated multisystem proteinopathy suggest altered mitochondrial bioenergetics.
Mitochondrion
; 22: 1-8, 2015 May.
Article
in English
| MEDLINE | ID: mdl-25724235
13.
Cytokine profiling in patients with VCP-associated disease.
Clin Transl Sci
; 7(1): 29-32, 2014 Feb.
Article
in English
| MEDLINE | ID: mdl-24119107
14.
Exercise training reverses skeletal muscle atrophy in an experimental model of VCP disease.
PLoS One
; 8(10): e76187, 2013.
Article
in English
| MEDLINE | ID: mdl-24130765
15.
The homozygote VCP(R¹55H/R¹55H) mouse model exhibits accelerated human VCP-associated disease pathology.
PLoS One
; 7(9): e46308, 2012.
Article
in English
| MEDLINE | ID: mdl-23029473
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