Primary coenzyme Q10 deficiency

L Salviati, E Trevisson, M Doimo, P Navas - GeneReviews, 2017 - research.unipd.it
CLINICAL CHARACTERISTICS: Primary coenzyme Q10 (CoQ10) deficiency is usually
associated with multisystem involvement, including neurologic manifestations such as fatal …

Clinical presentations of coenzyme q10 deficiency syndrome

CM Quinzii, V Emmanuele, M Hirano - Molecular syndromology, 2014 - karger.com
Abstract Coenzyme Q 10 (CoQ 10) deficiency is a clinically and genetically heterogeneous
syndrome which has been associated with 5 major clinical phenotypes:(1) …

Genetics of coenzyme q10 deficiency

M Doimo, MA Desbats, C Cerqua, M Cassina… - Molecular …, 2014 - karger.com
Abstract Coenzyme Q10 (CoQ10) is an essential component of eukaryotic cells and is
involved in crucial biochemical reactions such as the production of ATP in the mitochondrial …

Heterogeneity of coenzyme Q10 deficiency: patient study and literature review

V Emmanuele, LC López, A Berardo, A Naini… - Archives of …, 2012 - jamanetwork.com
Coenzyme Q 10 (CoQ 10) deficiency has been associated with 5 major clinical phenotypes:
encephalomyopathy, severe infantile multisystemic disease, nephropathy, cerebellar ataxia …

Biochemical Diagnosis of Coenzyme Q10 Deficiency

D Yubero, R Montero, R Artuch, JM Land… - Molecular …, 2014 - karger.com
Abstract Coenzyme Q10 (CoQ10) deficiency appears to have a particularly heterogeneous
clinical presentation. However, there appear to be 5 recognisable clinical phenotypes …

Clinical syndromes associated with Coenzyme Q10 deficiency

M Alcázar-Fabra, E Trevisson… - Essays in …, 2018 - portlandpress.com
Primary Coenzyme Q deficiencies represent a group of rare conditions caused by mutations
in one of the genes required in its biosynthetic pathway at the enzymatic or regulatory level …

176th ENMC International Workshop: diagnosis and treatment of coenzyme Q10 deficiency

S Rahman, CF Clarke, M Hirano - Neuromuscular disorders, 2012 - Elsevier
An ENMC meeting was held in Naarden, the Netherlands (July 9th–11th 2010) with 12
clinical and basic scientists and three industrial representatives from Europe (France …

Response to early coenzyme Q10 supplementation is not sustained in CoQ10 deficiency caused by CoQ2 mutation

FK Eroglu, F Ozaltin, N Gönç, H Nalçacıoğlu… - Pediatric …, 2018 - Elsevier
Background COQ2 mutations cause a rare infantile multisystemic disease with
heterogeneous clinical features. Promising results have been reported in response to …

A fatal case of COQ7‐associated primary coenzyme Q10 deficiency

AKY Kwong, ATG Chiu, MHY Tsang, KS Lun… - JIMD …, 2019 - Wiley Online Library
Abstract Background Primary coenzyme Q10 (CoQ10) deficiencies are clinically and
genetically heterogeneous group of disorders associated with defects of genes involved in …

Coenzyme Q10 is frequently reduced in muscle of patients with mitochondrial myopathy

S Sacconi, E Trevisson, L Salviati, S Aymé… - Neuromuscular …, 2010 - Elsevier
Coenzyme Q10 (CoQ10) deficiency has been associated with an increasing number of
clinical phenotypes. Whereas primary CoQ10 defects are related to mutations in ubiquinone …