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1.
Drug repositioning as a therapeutic strategy for neurodegenerations associated with OPA1 mutations.
Hum Mol Genet
; 29(22): 3631-3645, 2021 01 21.
Artículo
en Inglés
| MEDLINE | ID: mdl-33231680
2.
A Yeast-Based Repurposing Approach for the Treatment of Mitochondrial DNA Depletion Syndromes Led to the Identification of Molecules Able to Modulate the dNTP Pool.
Int J Mol Sci
; 22(22)2021 Nov 12.
Artículo
en Inglés
| MEDLINE | ID: mdl-34830106
3.
A Yeast-Based Screening Unravels Potential Therapeutic Molecules for Mitochondrial Diseases Associated with Dominant ANT1 Mutations.
Int J Mol Sci
; 22(9)2021 Apr 24.
Artículo
en Inglés
| MEDLINE | ID: mdl-33923309
4.
Defective mitochondrial rRNA methyltransferase MRM2 causes MELAS-like clinical syndrome.
Hum Mol Genet
; 26(21): 4257-4266, 2017 11 01.
Artículo
en Inglés
| MEDLINE | ID: mdl-28973171
5.
Recurrent De Novo Dominant Mutations in SLC25A4 Cause Severe Early-Onset Mitochondrial Disease and Loss of Mitochondrial DNA Copy Number.
Am J Hum Genet
; 99(4): 860-876, 2016 10 06.
Artículo
en Inglés
| MEDLINE | ID: mdl-27693233
6.
Sabotage at the Powerhouse? Unraveling the Molecular Target of 2-Isopropylbenzaldehyde Thiosemicarbazone, a Specific Inhibitor of Aflatoxin Biosynthesis and Sclerotia Development in Aspergillus flavus, Using Yeast as a Model System.
Molecules
; 24(16)2019 Aug 16.
Artículo
en Inglés
| MEDLINE | ID: mdl-31426298
7.
Combined use of Saccharomyces cerevisiae, Caenorhabditis elegans and patient fibroblasts leads to the identification of clofilium tosylate as a potential therapeutic chemical against POLG-related diseases.
Hum Mol Genet
; 25(4): 715-27, 2016 Feb 15.
Artículo
en Inglés
| MEDLINE | ID: mdl-26692522
8.
Biallelic Mutations of Methionyl-tRNA Synthetase Cause a Specific Type of Pulmonary Alveolar Proteinosis Prevalent on Réunion Island.
Am J Hum Genet
; 96(5): 826-31, 2015 May 07.
Artículo
en Inglés
| MEDLINE | ID: mdl-25913036
9.
Dominance of yeast aac2R96H and aac2R252G mutations, equivalent to pathological mutations in ant1, is due to gain of function.
Biochem Biophys Res Commun
; 493(2): 909-913, 2017 11 18.
Artículo
en Inglés
| MEDLINE | ID: mdl-28947214
10.
Structural modification of cuminaldehyde thiosemicarbazone increases inhibition specificity toward aflatoxin biosynthesis and sclerotia development in Aspergillus flavus.
Appl Microbiol Biotechnol
; 101(17): 6683-6696, 2017 Sep.
Artículo
en Inglés
| MEDLINE | ID: mdl-28725928
11.
Mitochondrial thiol oxidase Erv1: both shuttle cysteine residues are required for its function with distinct roles.
Biochem J
; 460(2): 199-210, 2014 Jun 01.
Artículo
en Inglés
| MEDLINE | ID: mdl-24625320
12.
Recurrent De Novo Dominant Mutations in SLC25A4 Cause Severe Early-Onset Mitochondrial Disease and Loss of Mitochondrial DNA Copy Number.
Am J Hum Genet
; 99(6): 1405, 2016 12 01.
Artículo
en Inglés
| MEDLINE | ID: mdl-27912046
13.
Zebrafish polg2 knock-out recapitulates human POLG-disorders; implications for drug treatment.
Cell Death Dis
; 15(4): 281, 2024 Apr 20.
Artículo
en Inglés
| MEDLINE | ID: mdl-38643274
14.
The mitochondrial disulfide relay system protein GFER is mutated in autosomal-recessive myopathy with cataract and combined respiratory-chain deficiency.
Am J Hum Genet
; 84(5): 594-604, 2009 May.
Artículo
en Inglés
| MEDLINE | ID: mdl-19409522
15.
FLO11 expression and lipid biosynthesis are required for air-liquid biofilm formation in a Saccharomyces cerevisiae flor strain.
FEMS Yeast Res
; 12(7): 864-6, 2012 Nov.
Artículo
en Inglés
| MEDLINE | ID: mdl-22805178
16.
Insights into physiological and genetic mupirocin susceptibility in bifidobacteria.
Appl Environ Microbiol
; 77(9): 3141-6, 2011 May.
Artículo
en Inglés
| MEDLINE | ID: mdl-21421794
17.
The Power of Yeast in Modelling Human Nuclear Mutations Associated with Mitochondrial Diseases.
Genes (Basel)
; 12(2)2021 02 20.
Artículo
en Inglés
| MEDLINE | ID: mdl-33672627
18.
Efficient clofilium tosylate-mediated rescue of POLG-related disease phenotypes in zebrafish.
Cell Death Dis
; 12(1): 100, 2021 01 19.
Artículo
en Inglés
| MEDLINE | ID: mdl-33469036
19.
Mechanistic insights on the mode of action of an antiproliferative thiosemicarbazone-nickel complex revealed by an integrated chemogenomic profiling study.
Sci Rep
; 10(1): 10524, 2020 06 29.
Artículo
en Inglés
| MEDLINE | ID: mdl-32601343
20.
Modeling of pathogenic variants of mitochondrial DNA polymerase: insight into the replication defects and implication for human disease.
Biochim Biophys Acta Gen Subj
; 1864(7): 129608, 2020 07.
Artículo
en Inglés
| MEDLINE | ID: mdl-32234506