Detalhe da pesquisa
1.
Diversity in the origins of proteostasis networks--a driver for protein function in evolution.
Nat Rev Mol Cell Biol
; 14(4): 237-48, 2013 Apr.
Artigo
em Inglês
| MEDLINE | ID: mdl-23463216
2.
Biological and chemical approaches to diseases of proteostasis deficiency.
Annu Rev Biochem
; 78: 959-91, 2009.
Artigo
em Inglês
| MEDLINE | ID: mdl-19298183
3.
Sterol O-Acyltransferase 1 (SOAT1): A Genetic Modifier of Niemann-Pick Disease, Type C1.
Int J Mol Sci
; 25(8)2024 Apr 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-38673803
4.
Costly mistakes: translational infidelity and protein homeostasis.
Cell
; 134(2): 204-6, 2008 Jul 25.
Artigo
em Inglês
| MEDLINE | ID: mdl-18662533
5.
Structural basis for cargo regulation of COPII coat assembly.
Cell
; 134(3): 474-84, 2008 Aug 08.
Artigo
em Inglês
| MEDLINE | ID: mdl-18692470
6.
Chemical and biological approaches synergize to ameliorate protein-folding diseases.
Cell
; 134(5): 769-81, 2008 Sep 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-18775310
7.
Individualized management of genetic diversity in Niemann-Pick C1 through modulation of the Hsp70 chaperone system.
Hum Mol Genet
; 29(1): 1-19, 2020 01 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-31509197
8.
Correction of Niemann-Pick type C1 trafficking and activity with the histone deacetylase inhibitor valproic acid.
J Biol Chem
; 295(23): 8017-8035, 2020 06 05.
Artigo
em Inglês
| MEDLINE | ID: mdl-32354745
9.
HDAC inhibitors rescue multiple disease-causing CFTR variants.
Hum Mol Genet
; 28(12): 1982-2000, 2019 06 15.
Artigo
em Inglês
| MEDLINE | ID: mdl-30753450
10.
∆F508 CFTR interactome remodelling promotes rescue of cystic fibrosis.
Nature
; 528(7583): 510-6, 2015 Dec 24.
Artigo
em Inglês
| MEDLINE | ID: mdl-26618866
11.
Correcting the F508del-CFTR variant by modulating eukaryotic translation initiation factor 3-mediated translation initiation.
J Biol Chem
; 293(35): 13477-13495, 2018 08 31.
Artigo
em Inglês
| MEDLINE | ID: mdl-30006345
12.
Silencing of the Hsp70-specific nucleotide-exchange factor BAG3 corrects the F508del-CFTR variant by restoring autophagy.
J Biol Chem
; 293(35): 13682-13695, 2018 08 31.
Artigo
em Inglês
| MEDLINE | ID: mdl-29986884
13.
Quantitative Analysis of the Proteome Response to the Histone Deacetylase Inhibitor (HDACi) Vorinostat in Niemann-Pick Type C1 disease.
Mol Cell Proteomics
; 16(11): 1938-1957, 2017 11.
Artigo
em Inglês
| MEDLINE | ID: mdl-28860124
14.
Histone deacetylase inhibitors correct the cholesterol storage defect in most Niemann-Pick C1 mutant cells.
J Lipid Res
; 58(4): 695-708, 2017 04.
Artigo
em Inglês
| MEDLINE | ID: mdl-28193631
15.
Modulation of the maladaptive stress response to manage diseases of protein folding.
PLoS Biol
; 12(11): e1001998, 2014 Nov.
Artigo
em Inglês
| MEDLINE | ID: mdl-25406061
16.
Quantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann-Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis.
Mol Cell Proteomics
; 14(7): 1734-49, 2015 Jul.
Artigo
em Inglês
| MEDLINE | ID: mdl-25873482
17.
Blue journal conference. Aging and susceptibility to lung disease.
Am J Respir Crit Care Med
; 191(3): 261-9, 2015 Feb 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-25590812
18.
Malfolded protein structure and proteostasis in lung diseases.
Am J Respir Crit Care Med
; 189(1): 96-103, 2014 Jan 01.
Artigo
em Inglês
| MEDLINE | ID: mdl-24033344
19.
Gelsolin amyloidosis: genetics, biochemistry, pathology and possible strategies for therapeutic intervention.
Crit Rev Biochem Mol Biol
; 47(3): 282-96, 2012.
Artigo
em Inglês
| MEDLINE | ID: mdl-22360545
20.
Quantitative proteomic profiling reveals differentially regulated proteins in cystic fibrosis cells.
J Proteome Res
; 13(11): 4668-75, 2014 Nov 07.
Artigo
em Inglês
| MEDLINE | ID: mdl-24818864