Product References
Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment.
Brain : a journal of neurology
Manolaras I,Del Bondio A,Griso O,Reutenauer L,Eisenmann A,Habermann BH,Puccio H
Fri Sep 01 00:00:00 EDT 2023
Mitochondrial dysfunction and calcium dysregulation in COQ8A-ataxia Purkinje neurons are rescued by CoQ10 treatment.
Brain : a journal of neurology
Manolaras I,Del Bondio A,Griso O,Reutenauer L,Eisenmann A,Habermann BH,Puccio H
Fri Sep 01 00:00:00 EDT 2023
The Q-junction and the inflammatory response are critical pathological and therapeutic factors in CoQ deficiency.
Redox biology
González-García P,Díaz-Casado ME,Hidalgo-Gutiérrez A,Jiménez-Sánchez L,Bakkali M,Barriocanal-Casado E,Escames G,Chiozzi RZ,Völlmy F,Zaal EA,Berkers CR,Heck AJR,López LC
Thu Sep 01 00:00:00 EDT 2022
Implication of folate deficiency in CYP2U1 loss of function.
The Journal of experimental medicine
Pujol C,Legrand A,Parodi L,Thomas P,Mochel F,Saracino D,Coarelli G,Croon M,Popovic M,Valet M,Villain N,Elshafie S,Issa M,Zuily S,Renaud M,Marelli-Tosi C,Legendre M,Trimouille A,Kemlin I,Mathieu S,Gleeson JG,Lamari F,Galatolo D,Alkouri R,Tse C,Rodriguez D,Ewenczyk C,Fellmann F,Kuntzer T,Blond E,El Hachimi KH,Darios F,Seyer A,Gazi AD,Giavalisco P,Perin S,Boucher JL,Le Corre L,Santorelli FM,Goizet C,Zaki MS,Picaud S,Mourier A,Steculorum SM,Mignot C,Durr A,Trifunovic A,Stevanin G
Mon Nov 01 00:00:00 EDT 2021
ADCK4 Deficiency Destabilizes the Coenzyme Q Complex, Which Is Rescued by 2,4-Dihydroxybenzoic Acid Treatment.
Journal of the American Society of Nephrology : JASN
Widmeier E,Yu S,Nag A,Chung YW,Nakayama M,Fernández-Del-Río L,Hugo H,Schapiro D,Buerger F,Choi WI,Helmstädter M,Kim JW,Ryu JH,Lee MG,Clarke CF,Hildebrandt F,Gee HY
Mon Jun 01 00:00:00 EDT 2020
β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9 mice.
EMBO molecular medicine
Hidalgo-Gutiérrez A,Barriocanal-Casado E,Bakkali M,Díaz-Casado ME,Sánchez-Maldonado L,Romero M,Sayed RK,Prehn C,Escames G,Duarte J,Acuña-Castroviejo D,López LC
Tue Jan 01 00:00:00 EST 2019
β-RA reduces DMQ/CoQ ratio and rescues the encephalopathic phenotype in Coq9 mice.
EMBO molecular medicine
Hidalgo-Gutiérrez A,Barriocanal-Casado E,Bakkali M,Díaz-Casado ME,Sánchez-Maldonado L,Romero M,Sayed RK,Prehn C,Escames G,Duarte J,Acuña-Castroviejo D,López LC
Tue Jan 01 00:00:00 EST 2019
Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis.
Scientific reports
Rodríguez-Hidalgo M,Luna-Sánchez M,Hidalgo-Gutiérrez A,Barriocanal-Casado E,Mascaraque C,Acuña-Castroviejo D,Rivera M,Escames G,López LC
Tue Sep 18 00:00:00 EDT 2018
Reduction in the levels of CoQ biosynthetic proteins is related to an increase in lifespan without evidence of hepatic mitohormesis.
Scientific reports
Rodríguez-Hidalgo M,Luna-Sánchez M,Hidalgo-Gutiérrez A,Barriocanal-Casado E,Mascaraque C,Acuña-Castroviejo D,Rivera M,Escames G,López LC
Tue Sep 18 00:00:00 EDT 2018
The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.
EMBO molecular medicine
Luna-Sánchez M,Díaz-Casado E,Barca E,Tejada MÁ,Montilla-García Á,Cobos EJ,Escames G,Acuña-Castroviejo D,Quinzii CM,López LC
Fri May 01 00:00:00 EDT 2015
The clinical heterogeneity of coenzyme Q10 deficiency results from genotypic differences in the Coq9 gene.
EMBO molecular medicine
Luna-Sánchez M,Díaz-Casado E,Barca E,Tejada MÁ,Montilla-García Á,Cobos EJ,Escames G,Acuña-Castroviejo D,Quinzii CM,López LC
Fri May 01 00:00:00 EDT 2015