Detalhe da pesquisa
1.
A Heterozygous NDUFV1 Variant Aggravates Mitochondrial Complex I Deficiency in a Family with a Homoplasmic ND1 Variant.
J Pediatr
; 196: 309-313.e3, 2018 05.
Artigo
Inglês
| MEDLINE | ID: mdl-29395179
2.
Mutations in COA6 cause cytochrome c oxidase deficiency and neonatal hypertrophic cardiomyopathy.
Hum Mutat
; 36(1): 34-8, 2015 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-25339201
3.
Solute diffusion is hindered in the mitochondrial matrix.
Proc Natl Acad Sci U S A
; 108(21): 8657-62, 2011 May 24.
Artigo
Inglês
| MEDLINE | ID: mdl-21555543
4.
Subunit-specific incorporation efficiency and kinetics in mitochondrial complex I homeostasis.
J Biol Chem
; 287(50): 41851-60, 2012 Dec 07.
Artigo
Inglês
| MEDLINE | ID: mdl-23038253
5.
Metabolic consequences of NDUFS4 gene deletion in immortalized mouse embryonic fibroblasts.
Biochim Biophys Acta
; 1817(10): 1925-36, 2012 Oct.
Artigo
Inglês
| MEDLINE | ID: mdl-22430089
6.
Effect of polymer composition on rheological and degradation properties of temperature-responsive gelling systems composed of acyl-capped PCLA-PEG-PCLA.
Biomacromolecules
; 14(9): 3172-82, 2013 Sep 09.
Artigo
Inglês
| MEDLINE | ID: mdl-23875877
7.
Assembly factors as a new class of disease genes for mitochondrial complex I deficiency: cause, pathology and treatment options.
Brain
; 135(Pt 1): 12-22, 2012 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-22036961
8.
TEFM (c17orf42) is necessary for transcription of human mtDNA.
Nucleic Acids Res
; 39(10): 4284-99, 2011 May.
Artigo
Inglês
| MEDLINE | ID: mdl-21278163
9.
Defective mitochondrial translation differently affects the live cell dynamics of complex I subunits.
Biochim Biophys Acta
; 1807(12): 1624-33, 2011 Dec.
Artigo
Inglês
| MEDLINE | ID: mdl-21978538
10.
Contiguous gene deletion of ELOVL7, ERCC8 and NDUFAF2 in a patient with a fatal multisystem disorder.
Hum Mol Genet
; 18(18): 3365-74, 2009 Sep 15.
Artigo
Inglês
| MEDLINE | ID: mdl-19525295
11.
A novel sustained-release cysteamine bitartrate formulation for the treatment of cystinosis: Pharmacokinetics and safety in healthy male volunteers.
Pharmacol Res Perspect
; 9(2): e00739, 2021 04.
Artigo
Inglês
| MEDLINE | ID: mdl-33764642
12.
First-in-Human Study of the Safety, Tolerability, Pharmacokinetics and - Preliminary Dynamics of Neuroprotectant 2-Iminobiotin in Healthy Subjects.
Curr Clin Pharmacol
; 15(2): 152-163, 2020.
Artigo
Inglês
| MEDLINE | ID: mdl-31625480
13.
TMEM70 functions in the assembly of complexes I and V.
Biochim Biophys Acta Bioenerg
; 1861(8): 148202, 2020 08 01.
Artigo
Inglês
| MEDLINE | ID: mdl-32275929
14.
Mitigation of NADH: ubiquinone oxidoreductase deficiency by chronic Trolox treatment.
Biochim Biophys Acta
; 1777(7-8): 853-9, 2008.
Artigo
Inglês
| MEDLINE | ID: mdl-18435906
15.
Human mitochondrial complex I assembly: a dynamic and versatile process.
Biochim Biophys Acta
; 1767(10): 1215-27, 2007 Oct.
Artigo
Inglês
| MEDLINE | ID: mdl-17854760
16.
Superoxide production is inversely related to complex I activity in inherited complex I deficiency.
Biochim Biophys Acta
; 1772(3): 373-81, 2007 Mar.
Artigo
Inglês
| MEDLINE | ID: mdl-17289351
17.
Novel controlled-release Lemna-derived IFN-alpha2b (Locteron): pharmacokinetics, pharmacodynamics, and tolerability in a phase I clinical trial.
J Interferon Cytokine Res
; 28(2): 113-22, 2008 Feb.
Artigo
Inglês
| MEDLINE | ID: mdl-18279106
18.
The effect of core composition in biodegradable oligomeric micelles as taxane formulations.
Eur J Pharm Biopharm
; 68(3): 596-606, 2008 Mar.
Artigo
Inglês
| MEDLINE | ID: mdl-17949956
19.
A mechanistic study on the chemical and enzymatic degradation of PEG-Oligo(epsilon-caprolactone) micelles.
J Pharm Sci
; 97(1): 506-18, 2008 Jan.
Artigo
Inglês
| MEDLINE | ID: mdl-17879976
20.
In vivo pharmacokinetics of celecoxib loaded endcapped PCLA-PEG-PCLA thermogels in rats after subcutaneous administration.
Eur J Pharm Biopharm
; 131: 170-177, 2018 Oct.
Artigo
Inglês
| MEDLINE | ID: mdl-30075312