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Secondary coenzyme Q(10) deficiencies in oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders

Fecha de publicación:

Autores de CIPF

Participantes ajenos a CIPF

  • Yubero, D
  • Montero, R
  • Martin, MA
  • Montoya, J
  • Ribes, A
  • Grazina, M
  • Trevisson, E
  • Rodriguez-Aguilera, JC
  • Hargreaves, LP
  • Salviati, L
  • Navas, P
  • Artuch, R
  • Jimenez-Mallebrera, C
  • Nascimento, A
  • Perez-Duenas, B
  • Ortez, C
  • Ramos, F
  • Colomer, J
  • O'Callaghan, M
  • Pineda, M
  • Garcia-Cazorla, A
  • Ruiz, A
  • Macaya, A
  • Marce-Grau, A
  • Garcia-Villoria, J
  • Arias, A
  • Emperador, S
  • Ruiz-Pesini, E
  • Lopez-Gallardo, E
  • Neergheen, V
  • Simoes, M
  • Diogo, L
  • Blazquez, A
  • Gonzalez-Quintana, A
  • Delmiro, A
  • Dominguez-Gonzalez, C
  • Arenas, J
  • Garcia-Silva, M
  • Martin, E
  • Quijada, P
  • Hernandez-Lain, A
  • Moran, M
  • Infante, ER
  • Polo, RA
  • Lope, CP
  • Lorite, JB
  • Fernandez, EM
  • Cortes, AB
  • Sanchez-Cuesta, A
  • Cascajo, MV
  • Alcazar, M
  • Brea-Calvo, G
  • CoQ Deficiency Study Grp Cristina

Grupos de Investigación

Abstract

We evaluated the coenzyme Q(10) (CoQ) levels inpatients who were diagnosed with mitochondrial oxidative phosphorylation (OXPHOS) and non-OXPHOS disorders (n = 72). Data from the 72 cases in this study revealed that 44.4% of patients showed low CoQ concentrations in either their skeletal muscle or skin fibroblasts. Our findings suggest that secondary CoQdeficiency is a common finding in OXPHOS and non-OXPHOS disorders. We hypothesize that cases of CoQdeficiency associated with OXPHOS defects could be an adaptive mechanism to maintain a balanced OXPHOS, although the mechanisms explaining these deficiencies and the pathophysiological role of secondary CoQ deficiency deserves further investigation. (C) 2016 Elsevier B.V. and Mitochondria Research Society. All rights reserved.

Datos de la publicación

ISSN/ISSNe:
1567-7249, 1872-8278

MITOCHONDRION  ELSEVIER SCI LTD

Tipo:
Article
Páginas:
51-58
PubMed:
27374853

Citas Recibidas en Web of Science: 52

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Keywords

  • Oxidative phosphorylation disorders; Coenzyme Q(10); Mitochondrial respiratory chain; Muscle biopsy

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