Detalhe da pesquisa
1.
Regulation of mitochondrial proteostasis by the proton gradient.
EMBO J
; 41(16): e110476, 2022 08 16.
Artigo
Inglês
| MEDLINE | ID: mdl-35912435
2.
AIFM1 is a component of the mitochondrial disulfide relay that drives complex I assembly through efficient import of NDUFS5.
EMBO J
; 41(17): e110784, 2022 09 01.
Artigo
Inglês
| MEDLINE | ID: mdl-35859387
3.
Ablation of mitochondrial DNA results in widespread remodeling of the mitochondrial complexome.
EMBO J
; 40(21): e108648, 2021 11 02.
Artigo
Inglês
| MEDLINE | ID: mdl-34542926
4.
Comparative Clustering (CompaCt) of eukaryote complexomes identifies novel interactions and sheds light on protein complex evolution.
PLoS Comput Biol
; 19(8): e1011090, 2023 08.
Artigo
Inglês
| MEDLINE | ID: mdl-37549177
5.
The m-AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria.
Mol Cell
; 64(1): 148-162, 2016 10 06.
Artigo
Inglês
| MEDLINE | ID: mdl-27642048
6.
Molecular characterization of a complex of apoptosis-inducing factor 1 with cytochrome c oxidase of the mitochondrial respiratory chain.
Proc Natl Acad Sci U S A
; 118(39)2021 09 28.
Artigo
Inglês
| MEDLINE | ID: mdl-34548399
7.
Bi-allelic Mutations in the Mitochondrial Ribosomal Protein MRPS2 Cause Sensorineural Hearing Loss, Hypoglycemia, and Multiple OXPHOS Complex Deficiencies.
Am J Hum Genet
; 102(4): 685-695, 2018 04 05.
Artigo
Inglês
| MEDLINE | ID: mdl-29576219
8.
Mutations in ATP6V1E1 or ATP6V1A Cause Autosomal-Recessive Cutis Laxa.
Am J Hum Genet
; 100(2): 216-227, 2017 02 02.
Artigo
Inglês
| MEDLINE | ID: mdl-28065471
9.
COmplexome Profiling ALignment (COPAL) reveals remodeling of mitochondrial protein complexes in Barth syndrome.
Bioinformatics
; 35(17): 3083-3091, 2019 09 01.
Artigo
Inglês
| MEDLINE | ID: mdl-30649188
10.
Novel defect in phosphatidylinositol 4-kinase type 2-alpha (PI4K2A) at the membrane-enzyme interface is associated with metabolic cutis laxa.
J Inherit Metab Dis
; 43(6): 1382-1391, 2020 11.
Artigo
Inglês
| MEDLINE | ID: mdl-32418222
11.
Mutations in Complex I Assembly Factor TMEM126B Result in Muscle Weakness and Isolated Complex I Deficiency.
Am J Hum Genet
; 99(1): 208-16, 2016 Jul 07.
Artigo
Inglês
| MEDLINE | ID: mdl-27374773
12.
Mutations in ATP6V1E1 or ATP6V1A Cause Autosomal-Recessive Cutis Laxa.
Am J Hum Genet
; 107(2): 374, 2020 08 06.
Artigo
Inglês
| MEDLINE | ID: mdl-32763190
13.
The membrane scaffold SLP2 anchors a proteolytic hub in mitochondria containing PARL and the i-AAA protease YME1L.
EMBO Rep
; 17(12): 1844-1856, 2016 12.
Artigo
Inglês
| MEDLINE | ID: mdl-27737933
14.
Compound heterozygosity for severe and hypomorphic NDUFS2 mutations cause non-syndromic LHON-like optic neuropathy.
J Med Genet
; 54(5): 346-356, 2017 05.
Artigo
Inglês
| MEDLINE | ID: mdl-28031252
15.
Accessory NUMM (NDUFS6) subunit harbors a Zn-binding site and is essential for biogenesis of mitochondrial complex I.
Proc Natl Acad Sci U S A
; 112(18): 5685-90, 2015 May 05.
Artigo
Inglês
| MEDLINE | ID: mdl-25902503
16.
Biallelic variants in WARS2 encoding mitochondrial tryptophanyl-tRNA synthase in six individuals with mitochondrial encephalopathy.
Hum Mutat
; 38(12): 1786-1795, 2017 12.
Artigo
Inglês
| MEDLINE | ID: mdl-28905505
17.
Structure and function of mitochondrial complex I.
Biochim Biophys Acta
; 1857(7): 902-14, 2016 Jul.
Artigo
Inglês
| MEDLINE | ID: mdl-26921811
18.
Evolution and structural organization of the mitochondrial contact site (MICOS) complex and the mitochondrial intermembrane space bridging (MIB) complex.
Biochim Biophys Acta
; 1863(1): 91-101, 2016 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-26477565
19.
Cryo-EM structure of respiratory complex I reveals a link to mitochondrial sulfur metabolism.
Biochim Biophys Acta
; 1857(12): 1935-1942, 2016 12.
Artigo
Inglês
| MEDLINE | ID: mdl-27693469
20.
The LYR protein subunit NB4M/NDUFA6 of mitochondrial complex I anchors an acyl carrier protein and is essential for catalytic activity.
Proc Natl Acad Sci U S A
; 111(14): 5207-12, 2014 Apr 08.
Artigo
Inglês
| MEDLINE | ID: mdl-24706851