Product References
Homozygous COQ7 mutation: a new cause of potentially treatable distal hereditary motor neuropathy.
Brain : a journal of neurology
Jacquier A,Theuriet J,Fontaine F,Mosbach V,Lacoste N,Ribault S,Risson V,Carras J,Coudert L,Simonet T,Latour P,Stojkovic T,Piard J,Cosson A,Lesca G,Bouhour F,Allouche S,Puccio H,Pegat A,Schaeffer L
Tue Aug 01 00:00:00 UTC 2023
Novel Homozygous Variant in COQ7 in Siblings With Hereditary Motor Neuropathy.
Neurology. Genetics
Smith IC,Pileggi CA,Wang Y,Kernohan K,Hartley T,McMillan HJ,Sampaio ML,Melkus G,Woulfe J,Parmar G,Bourque PR,Breiner A,Zwicker J,Pringle CE,Jarinova O,Lochmüller H,Dyment DA,Brais B,Boycott KM,Hekimi S,Harper ME,Warman-Chardon J
Wed Feb 01 00:00:00 UTC 2023
The CoQ biosynthetic di-iron carboxylate hydroxylase COQ7 is inhibited by in vivo metalation with manganese but remains functional by metalation with cobalt.
microPublication biology
Wang Y,Hekimi S
Sat Oct 01 00:00:00 UTC 2022
A novel COQ7 mutation causing primarily neuromuscular pathology and its treatment options.
Molecular genetics and metabolism reports
Wang Y,Gumus E,Hekimi S
Wed Jun 01 00:00:00 UTC 2022
Human COQ4 deficiency: delineating the clinical, metabolic and neuroimaging phenotypes.
Journal of medical genetics
Laugwitz L,Seibt A,Herebian D,Peralta S,Kienzle I,Buchert R,Falb R,Gauck D,Müller A,Grimmel M,Beck-Woedel S,Kern J,Daliri K,Katibeh P,Danhauser K,Leiz S,Alesi V,Baertling F,Vasco G,Steinfeld R,Wagner M,Caglayan AO,Gumus H,Burmeister M,Mayatepek E,Martinelli D,Tamhankar PM,Tamhankar V,Joset P,Steindl K,Rauch A,Bonnen PE,Froukh T,Groeschel S,Krägeloh-Mann I,Haack TB,Distelmaier F
Thu Sep 01 00:00:00 UTC 2022
The complex formation of MASP-3 with pattern recognition molecules of the lectin complement pathway retains MASP-3 in the circulation.
Frontiers in immunology
Kusakari K,Machida T,Ishida Y,Omori T,Suzuki T,Sekimata M,Wada I,Fujita T,Sekine H
Thu Sep 08 00:00:00 UTC 2022
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 UTC 2022
Dysregulation of iron homeostasis and methamphetamine reward behaviors in Clk1-deficient mice.
Acta pharmacologica Sinica
Yan PJ,Ren ZX,Shi ZF,Wan CL,Han CJ,Zhu LS,Li NN,Waddington JL,Zhen XC
Fri Jul 01 00:00:00 UTC 2022
Cold-inducible RNA binding protein agonist enhances the cardioprotective effect of UW solution during extended heart preservation.
Artificial organs
Pan HZ,Zhang LJ,Liu YW,Li YN,Su ZH,Meng J,Zhang H
Thu Oct 01 00:00:00 UTC 2020
Resveratrol Regulates the Expression of Genes Involved in CoQ Synthesis in Liver in Mice Fed with High Fat Diet.
Antioxidants (Basel, Switzerland)
Meza-Torres C,Hernández-Camacho JD,Cortés-Rodríguez AB,Fang L,Bui Thanh T,Rodríguez-Bies E,Navas P,López-Lluch G
Fri May 15 00:00:00 UTC 2020
Hepatic HuR modulates lipid homeostasis in response to high-fat diet.
Nature communications
Zhang Z,Zong C,Jiang M,Hu H,Cheng X,Ni J,Yi X,Jiang B,Tian F,Chang MW,Su W,Zhu L,Li J,Xiang X,Miao C,Gorospe M,de Cabo R,Dou Y,Ju Z,Yang J,Jiang C,Yang Z,Wang W
Tue Jun 16 00:00:00 UTC 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 UTC 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 UTC 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 UTC 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 UTC 2018
Clk1-regulated aerobic glycolysis is involved in glioma chemoresistance.
Journal of neurochemistry
Zhang L,Yang H,Zhang W,Liang Z,Huang Q,Xu G,Zhen X,Zheng LT
Tue Aug 01 00:00:00 UTC 2017
Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.
Molecular genetics and metabolism
Herebian D,Seibt A,Smits SHJ,Bünning G,Freyer C,Prokisch H,Karall D,Wredenberg A,Wedell A,López LC,Mayatepek E,Distelmaier F
Sat Jul 01 00:00:00 UTC 2017
Detection of 6-demethoxyubiquinone in CoQ10 deficiency disorders: Insights into enzyme interactions and identification of potential therapeutics.
Molecular genetics and metabolism
Herebian D,Seibt A,Smits SHJ,Bünning G,Freyer C,Prokisch H,Karall D,Wredenberg A,Wedell A,López LC,Mayatepek E,Distelmaier F
Sat Jul 01 00:00:00 UTC 2017
Activation of AMPK/mTORC1-Mediated Autophagy by Metformin Reverses Clk1 Deficiency-Sensitized Dopaminergic Neuronal Death.
Molecular pharmacology
Yan Q,Han C,Wang G,Waddington JL,Zheng L,Zhen X
Fri Dec 01 00:00:00 UTC 2017
Activation of AMPK/mTORC1-Mediated Autophagy by Metformin Reverses Clk1 Deficiency-Sensitized Dopaminergic Neuronal Death.
Molecular pharmacology
Yan Q,Han C,Wang G,Waddington JL,Zheng L,Zhen X
Fri Dec 01 00:00:00 UTC 2017
4-Hydroxybenzoic acid restores CoQ10 biosynthesis in human COQ2 deficiency.
Annals of clinical and translational neurology
Herebian D,Seibt A,Smits SHJ,Rodenburg RJ,Mayatepek E,Distelmaier F
Fri Dec 01 00:00:00 UTC 2017
Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9.
European journal of human genetics : EJHG
Danhauser K,Herebian D,Haack TB,Rodenburg RJ,Strom TM,Meitinger T,Klee D,Mayatepek E,Prokisch H,Distelmaier F
Tue Mar 01 00:00:00 UTC 2016
Fatal neonatal encephalopathy and lactic acidosis caused by a homozygous loss-of-function variant in COQ9.
European journal of human genetics : EJHG
Danhauser K,Herebian D,Haack TB,Rodenburg RJ,Strom TM,Meitinger T,Klee D,Mayatepek E,Prokisch H,Distelmaier F
Tue Mar 01 00:00:00 UTC 2016
A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
Nature cell biology
Monaghan RM,Barnes RG,Fisher K,Andreou T,Rooney N,Poulin GB,Whitmarsh AJ
Mon Jun 01 00:00:00 UTC 2015
A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
Nature cell biology
Monaghan RM,Barnes RG,Fisher K,Andreou T,Rooney N,Poulin GB,Whitmarsh AJ
Mon Jun 01 00:00:00 UTC 2015
A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
Nature cell biology
Monaghan RM,Barnes RG,Fisher K,Andreou T,Rooney N,Poulin GB,Whitmarsh AJ
Mon Jun 01 00:00:00 UTC 2015
A nuclear role for the respiratory enzyme CLK-1 in regulating mitochondrial stress responses and longevity.
Nature cell biology
Monaghan RM,Barnes RG,Fisher K,Andreou T,Rooney N,Poulin GB,Whitmarsh AJ
Mon Jun 01 00:00:00 UTC 2015
ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.
The Journal of clinical investigation
Ashraf S,Gee HY,Woerner S,Xie LX,Vega-Warner V,Lovric S,Fang H,Song X,Cattran DC,Avila-Casado C,Paterson AD,Nitschké P,Bole-Feysot C,Cochat P,Esteve-Rudd J,Haberberger B,Allen SJ,Zhou W,Airik R,Otto EA,Barua M,Al-Hamed MH,Kari JA,Evans J,Bierzynska A,Saleem MA,Böckenhauer D,Kleta R,El Desoky S,Hacihamdioglu DO,Gok F,Washburn J,Wiggins RC,Choi M,Lifton RP,Levy S,Han Z,Salviati L,Prokisch H,Williams DS,Pollak M,Clarke CF,Pei Y,Antignac C,Hildebrandt F
Sun Dec 01 00:00:00 UTC 2013
ADCK4 mutations promote steroid-resistant nephrotic syndrome through CoQ10 biosynthesis disruption.
The Journal of clinical investigation
Ashraf S,Gee HY,Woerner S,Xie LX,Vega-Warner V,Lovric S,Fang H,Song X,Cattran DC,Avila-Casado C,Paterson AD,Nitschké P,Bole-Feysot C,Cochat P,Esteve-Rudd J,Haberberger B,Allen SJ,Zhou W,Airik R,Otto EA,Barua M,Al-Hamed MH,Kari JA,Evans J,Bierzynska A,Saleem MA,Böckenhauer D,Kleta R,El Desoky S,Hacihamdioglu DO,Gok F,Washburn J,Wiggins RC,Choi M,Lifton RP,Levy S,Han Z,Salviati L,Prokisch H,Williams DS,Pollak M,Clarke CF,Pei Y,Antignac C,Hildebrandt F
Sun Dec 01 00:00:00 UTC 2013