Daniel J M Fernandez-Ayala
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Explore the profile of Daniel J M Fernandez-Ayala including associated specialties, affiliations and a list of published articles.
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374
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Recent Articles
1.
Fernandez-Ayala D, Jimenez-Gancedo S, Guerra I, Hernandez-Camacho J, Neto M, Scialo F, et al.
Free Radic Biol Med
. 2025 Jan;
230:95-111.
PMID: 39864756
The interference of the expression of each of the genes involved in the synthesis of coenzyme Q (CoQ) in Drosophila melanogaster can help to understand the pathophysiology of CoQ-dependent mitochondrial...
2.
Alcazar-Fabra M, Ostergaard E, Fernandez-Ayala D, Desbats M, Morbidoni V, Tomas-Gallado L, et al.
Mol Genet Metab Rep
. 2025 Jan;
42:101176.
PMID: 39759098
Background And Aims: Primary Coenzyme Q (CoQ) deficiency caused by defects is a clinically heterogeneous mitochondrial condition characterized by reduced levels of CoQ in tissues. Next-generation sequencing has lately boosted...
3.
Hernandez-Camacho J, Vicente-Garcia C, Ardila-Garcia L, Padilla-Campos A, Lopez-Lluch G, Santos-Ocana C, et al.
J Cachexia Sarcopenia Muscle
. 2024 Oct;
15(6):2402-2416.
PMID: 39354863
Background: ADCK genes encode aarF domain-containing mitochondrial kinases involved in coenzyme Q (CoQ) biosynthesis and regulation. Haploinsufficiency of ADCK2 in humans leads to adult-onset physical incapacity with reduced mitochondrial CoQ...
4.
Hernandez-Camacho J, Fernandez-Ayala D, Vicente-Garcia C, Navas-Enamorado I, Lopez-Lluch G, Oliva C, et al.
Front Physiol
. 2022 Aug;
13:898792.
PMID: 35936917
ADCK2 haploinsufficiency-mediated mitochondrial coenzyme Q deficiency in skeletal muscle causes mitochondrial myopathy associated with defects in beta-oxidation of fatty acids, aged-matched metabolic reprogramming, and defective physical performance. Calorie restriction has...
5.
Hernandez-Camacho J, Vicente-Garcia C, Sanchez-Cuesta A, Fernandez-Ayala D, Carvajal J, Navas P
J Vis Exp
. 2022 Feb;
(180).
PMID: 35225269
Most of the cell's energy is obtained through the degradation of glucose, fatty acids, and amino acids by different pathways that converge on the mitochondrial oxidative phosphorylation (OXPHOS) system, which...
6.
Navas P, Cascajo M, Alcazar-Fabra M, Hernandez-Camacho J, Sanchez-Cuesta A, Rodriguez A, et al.
Biofactors
. 2021 Apr;
47(4):551-569.
PMID: 33878238
Coenzyme Q (CoQ ) deficiency is a rare disease characterized by a decreased accumulation of CoQ in cell membranes. Considering that CoQ synthesis and most of its functions are carried...
7.
Vazquez-Fonseca L, Schaefer J, Navas-Enamorado I, Santos-Ocana C, Hernandez-Camacho J, Guerra I, et al.
J Clin Med
. 2019 Sep;
8(9).
PMID: 31480808
Fatty acids and glucose are the main bioenergetic substrates in mammals. Impairment of mitochondrial fatty acid oxidation causes mitochondrial myopathy leading to decreased physical performance. Here, we report that haploinsufficiency...
8.
Rodriguez-Aguilera J, Cortes A, Fernandez-Ayala D, Navas P
J Clin Med
. 2017 Mar;
6(3).
PMID: 28273876
Coenzyme Q (CoQ) deficiency syndrome includes clinically heterogeneous mitochondrial diseases that show a variety of severe and debilitating symptoms. A multiprotein complex encoded by nuclear genes carries out CoQ biosynthesis....
9.
Cascajo M, Abdelmohsen K, Noh J, Fernandez-Ayala D, Willers I, Brea G, et al.
RNA Biol
. 2015 Dec;
13(7):622-34.
PMID: 26690054
Coenzyme Q (CoQ) is a key component of the mitochondrial respiratory chain carrying electrons from complexes I and II to complex III and it is an intrinsic component of the...
10.
Fernandez-Ayala D, Jimenez-Gancedo S, Guerra I, Navas P
Mol Syndromol
. 2014 Aug;
5(3-4):170-9.
PMID: 25126050
The human syndrome of coenzyme Q (CoQ) deficiency is a heterogeneous mitochondrial disease characterized by a diminution of CoQ content in cells and tissues that affects all the electron transport...