Identification of drug candidates which increase cytochrome c oxidase activity in deficient patient fibroblasts

被引：5

作者：

Maj, Mary ^{[1
,2
]}

Sriskandarajah, Niroshan ^{[1
]}

Hung, Vinci ^{[1
]}

Browne, Ikennah ^{[1
]}

Shah, Bhavank ^{[1
]}

Weadge, Anita ^{[1
]}

Jamieson, Nicola L. ^{[1
]}

Tropak, Michael ^{[3
]}

Cameron, Jessie M. ^{[1
]}

Addis, Jane B. ^{[1
]}

Robinson, Brian H. ^{[1
,4
,5
]}

机构：

[1] Hosp Sick Children, Res Inst, Metab Res Programme, Toronto, ON M5G 1X8, Canada

[2] Hosp Sick Children, Dept Paediat Lab Med, Toronto, ON M5G 1X8, Canada

[3] Hosp Sick Children, Res Inst, Lysosomal Res Programme, Toronto, ON M5G 1X8, Canada

[4] Univ Toronto, Dept Biochem, Toronto, ON M5S 1A8, Canada

[5] Univ Toronto, Dept Paediat, Toronto, ON M5S 1A8, Canada

来源：

MITOCHONDRION | 2011年 / 11卷 / 02期

关键词：

Cytochrome c oxidase; Mitochondria; High-throughput; Live cell assay; Chemical library screen; MITOCHONDRIAL RESPIRATORY-CHAIN; CELL-LINES; ACETYLATION; PGC-1-ALPHA; ACTIVATION; DEFECTS; ROSIGLITAZONE; BIOGENESIS; PROTEINS; BETULIN;

D O I：

10.1016/j.mito.2010.10.002

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

Cytochrome c oxidase (COX) activity reflects the expressed level of respiratory chain complexes, mtDNA levels, titer and mass of mitochondria. Activity is also indicative of the overall fitness of mt-transcription factors and the import, transcription and translation of mt-proteins. We have developed a high-throughput assay to measure COX activity using live cells to screen chemical libraries for compounds capable of increasing COX activity. These libraries have revealed four examples which elevated the activities of COX in NIH-3T3 fibroblasts and in fibroblasts from patients with COX defects independent of the peroxisome proliferator activated receptor family. (C) 2010 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

引用

页码：264 / 272

页数：9

共 34 条

[1] Activation of peroxisome proliferator-activated receptor pathway stimulates the mitochondrial respiratory chain and can correct deficiencies in patients' cells lacking its components
Bastin, Jean
Aubey, Flore
Rotig, Agnes
Munnich, Arnold
Djouadi, Fatima
[J]. JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM, 2008, 93 (04) : 1433 - 1441
[2] HISTOCHEMICAL DEMONSTRATION OF CYTOCHROME OXIDASE WITH NEW AMINE REAGENTS
BURSTONE, MS
[J]. JOURNAL OF HISTOCHEMISTRY & CYTOCHEMISTRY, 1960, 8 (01) : 63 - 70
[3] NEW HISTOCHEMICAL TECHNIQUES FOR THE DEMONSTRATION OF TISSUE OXIDASE (CYTOCHROME OXIDASE)
BURSTONE, MS
[J]. JOURNAL OF HISTOCHEMISTRY & CYTOCHEMISTRY, 1959, 7 (02) : 112 - 122
[4] HISTOCHEMICAL LOCALIZATION OF OXIDASE ACTIVITY IN THE MITOCHONDRIA OF THE HUMAN HEART
BURSTONE, MS
[J]. NATURE, 1959, 184 (4684) : 476 - 477
[5] A MICROTITER PLATE ASSAY FOR CYTOCHROME-C-OXIDASE IN PERMEABILIZED WHOLE CELLS
CHRZANOWSKALIGHTOWLERS, ZMA
TURNBULL, DM
LIGHTOWLERS, RN
[J]. ANALYTICAL BIOCHEMISTRY, 1993, 214 (01) : 45 - 49
[6] cAMP/Ca2+ Response Element-Binding Protein Plays a Central Role in the Biogenesis of Respiratory Chain Proteins in Mammalian Cells
De Rasmo, Domenico
Signorile, Anna
Papa, Francesco
Roca, Emilio
Papa, Sergio
[J]. IUBMB LIFE, 2010, 62 (06) : 447 - 452
[7] Mitochondrial diseases
DiMauro, S
[J]. BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS, 2004, 1658 (1-2): : 80 - 88
[8] Novel genetically encoded biosensors using firefly luciferase
Fan, Frank
Binkowski, Brock F.
Butler, Braeden L.
Stecha, Peter F.
Lewils, Martin K.
Wood, Keith V.
[J]. ACS CHEMICAL BIOLOGY, 2008, 3 (06) : 346 - 351
[9] Transcriptional coregulators in the control of energy homeostasis
Feige, Jerome N.
Auwerx, Johan
[J]. TRENDS IN CELL BIOLOGY, 2007, 17 (06) : 292 - 301
[10] GLERUM M, 1987, AM J HUM GENET, V41, P584

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