|
|
|
|
| |
|
|
| Fragaki,Konstantina; Chaussenot,Annabelle; Benoist,Jean-François; Ait-El-Mkadem,Samira; Bannwarth,Sylvie; Rouzier,Cécile; Cochaud,Charlotte; Paquis-Flucklinger,Véronique. |
| BACKGROUND: Coenzyme Q10 (CoQ10 or ubiquinone) deficiency can be due either to mutations in genes involved in CoQ10 biosynthesis pathway, or to mutations in genes unrelated to CoQ10 biosynthesis. CoQ10 defect is the only oxidative phosphorylation disorder that can be clinically improved after oral CoQ10 supplementation. Thus, early diagnosis, first evoked by mitochondrial respiratory chain (MRC) spectrophotometric analysis, then confirmed by direct measurement of CoQ10 levels, is of critical importance to prevent irreversible damage in organs such as the kidney and the central nervous system. It is widely reported that CoQ10 deficient patients present decreased quinone-dependent activities (segments I + III or G3P + III and II + III) while MRC activities... |
| Tipo: Journal article |
Palavras-chave: Mitochondrial disease; CoQ10 deficiency; Respiratory chain; Spectrophotometry; LC-MSMS. |
| Ano: 2016 |
URL: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0716-97602016000100004 |
| |
|
|
| Mariano,André Bellin; Kovalhuk,Leonardo; Valente,Caroline; Maurer-Menestrina,Juliana; Pereira-Netto,Adaucto Bellarmino; Guerra,Miguel Pedro; Carnieri,Eva Gunilla Skare. |
| A method for the isolation of coupled mitochondria from the callus of Araucaria angustifolia is described for the first time. Mitochondria were isolated from embryogenic callus of A. angustifolia. They were metabolically active, able to sustain oxidative phosphorylation as shown by respiratory control ratio values, which were about 2.4 when respiring on succinate as substrate. Oxygen uptake experiments, using freeze-thawed disrupted mitochondria, showed the presence of alternative rotenone-insensitive NAD(P)H dehydrogenases, which were stimulated by Ca2+. The procedure now described for the isolation of A. angustifolia mitochondria is an important new tool, allowing the investigation of mitochondrial bioenergetics and metabolism and physiology of plants. |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Araucaria angustifolia; Callus; Plant mitochondria; Respiratory chain. |
| Ano: 2004 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S1516-89132004000600006 |
| |
|
|
| Cavalheiro,R.A.; Fortes,F.; Borecký,J.; Faustinoni,V.C.; Schreiber,A.Z.. |
| The respiration, membrane potential (<FONT FACE=Symbol>Dy</FONT>), and oxidative phosphorylation of mitochondria in situ were determined in spheroplasts obtained from Candida albicans control strain ATCC 90028 by lyticase treatment. Mitochondria in situ were able to phosphorylate externally added ADP (200 µM) in the presence of 0.05% BSA. Mitochondria in situ generated and sustained stable mitochondrial <FONT FACE=Symbol>Dy</FONT> respiring on 5 mM NAD-linked substrates, 5 mM succinate, or 100 µM N,N,N',N'-tetramethyl-p-phenylenediamine dihydrochloride plus 1 mM ascorbate. Rotenone (4 µM) inhibited respiration by 30% and 2 µM antimycin A or myxothiazole and 1 mM cyanide inhibited it by 85%. Cyanide-insensitive respiration was... |
| Tipo: Info:eu-repo/semantics/article |
Palavras-chave: Candida albicans spheroplasts; Mitochondria; Respiratory chain; Mitochondrial membrane potential; Uncoupling protein. |
| Ano: 2004 |
URL: http://www.scielo.br/scielo.php?script=sci_arttext&pid=S0100-879X2004001000003 |
| |
|
|
|
