Detalhe da pesquisa
1.
Mitochondrial dysfunction caused by novel ATAD3A mutations.
Mol Genet Metab
; 131(1-2): 107-113, 2020.
Artigo
Inglês
| MEDLINE | ID: mdl-32933822
2.
Dymeclin deficiency causes postnatal microcephaly, hypomyelination and reticulum-to-Golgi trafficking defects in mice and humans.
Hum Mol Genet
; 24(10): 2771-83, 2015 May 15.
Artigo
Inglês
| MEDLINE | ID: mdl-25652408
3.
Mutations in CYC1, encoding cytochrome c1 subunit of respiratory chain complex III, cause insulin-responsive hyperglycemia.
Am J Hum Genet
; 93(2): 384-9, 2013 Aug 08.
Artigo
Inglês
| MEDLINE | ID: mdl-23910460
4.
Cis-silencing of PIP5K1B evidenced in Friedreich's ataxia patient cells results in cytoskeleton anomalies.
Hum Mol Genet
; 22(14): 2894-904, 2013 Jul 15.
Artigo
Inglês
| MEDLINE | ID: mdl-23552101
5.
Mitochondrial proteases and cancer.
Biochim Biophys Acta
; 1807(6): 595-601, 2011 Jun.
Artigo
Inglês
| MEDLINE | ID: mdl-21194520
6.
Identification of novel oxidized protein substrates and physiological partners of the mitochondrial ATP-dependent Lon-like protease Pim1.
J Biol Chem
; 285(15): 11445-57, 2010 Apr 09.
Artigo
Inglês
| MEDLINE | ID: mdl-20150421
7.
The NDUFB6 subunit of the mitochondrial respiratory chain complex I is required for electron transfer activity: a proof of principle study on stable and controlled RNA interference in human cell lines.
Biochem Biophys Res Commun
; 414(2): 367-72, 2011 Oct 22.
Artigo
Inglês
| MEDLINE | ID: mdl-21964293
8.
Friedreich's ataxia: the vicious circle hypothesis revisited.
BMC Med
; 9: 112, 2011 Oct 11.
Artigo
Inglês
| MEDLINE | ID: mdl-21985033
9.
Induced pluripotent stem cells-derived neurons from patients with Friedreich ataxia exhibit differential sensitivity to resveratrol and nicotinamide.
Sci Rep
; 9(1): 14568, 2019 10 10.
Artigo
Inglês
| MEDLINE | ID: mdl-31601825
10.
Towards the control of intracellular protein turnover: mitochondrial Lon protease inhibitors versus proteasome inhibitors.
Biochimie
; 90(2): 260-9, 2008 Feb.
Artigo
Inglês
| MEDLINE | ID: mdl-18021745
11.
FAST-1 antisense RNA epigenetically alters FXN expression.
Sci Rep
; 8(1): 17217, 2018 11 21.
Artigo
Inglês
| MEDLINE | ID: mdl-30464193
12.
Large Interruptions of GAA Repeat Expansion Mutations in Friedreich Ataxia Are Very Rare.
Front Cell Neurosci
; 12: 443, 2018.
Artigo
Inglês
| MEDLINE | ID: mdl-30519163
13.
Effects of Lon protease down-regulation on the mitochondrial function and proteome.
Free Radic Biol Med
; 75 Suppl 1: S32-3, 2014 Oct.
Artigo
Inglês
| MEDLINE | ID: mdl-26461341
14.
Effect of Lon protease knockdown on mitochondrial function in HeLa cells.
Biochimie
; 100: 38-47, 2014 May.
Artigo
Inglês
| MEDLINE | ID: mdl-24355201
15.
Friedreich's ataxia, frataxin, PIP5K1B: echo of a distant fracas.
Oxid Med Cell Longev
; 2013: 725635, 2013.
Artigo
Inglês
| MEDLINE | ID: mdl-24194977
16.
Deletion of the mitochondrial Pim1/Lon protease in yeast results in accelerated aging and impairment of the proteasome.
Free Radic Biol Med
; 56: 9-16, 2013 Mar.
Artigo
Inglês
| MEDLINE | ID: mdl-23220263
17.
The TOMM machinery is a molecular switch in PINK1 and PARK2/PARKIN-dependent mitochondrial clearance.
Autophagy
; 9(11): 1801-17, 2013 Nov 01.
Artigo
Inglês
| MEDLINE | ID: mdl-24149440
18.
Overexpression of the yeast frataxin homolog (Yfh1): contrasting effects on iron-sulfur cluster assembly, heme synthesis and resistance to oxidative stress.
Mitochondrion
; 9(2): 130-8, 2009 Apr.
Artigo
Inglês
| MEDLINE | ID: mdl-19460301