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Lionel Van Maldergem et al.
Annals of neurology, 52(6), 750-754 (2002-11-26)
A 31-year-old woman had encephalopathy, growth retardation, infantilism, ataxia, deafness, lactic acidosis, and increased signals of caudate and putamen on brain magnetic resonance imaging. Muscle biochemistry showed succinate:cytochrome c oxidoreductase (complex II-III) deficiency. Both clinical and biochemical abnormalities improved remarkably...
Elísabet Alcocer-Gómez et al.
Nutrition (Burbank, Los Angeles County, Calif.), 29(11-12), 1422-1425 (2013-10-10)
Recently, Coenzyme Q10 (CoQ10) deficiency has been implicated in the pathophysiology of fibromyalgia (FM). It is our objective to present the findings of the FM evaluation before and after oral CoQ10 treatment using the American College of Rheumatology (ACR) Diagnostic...
D Orsucci et al.
Current medicinal chemistry, 18(26), 4053-4064 (2011-08-10)
Coenzyme Q10 is a small electron carrier of the respiratory chain with antioxidant properties, widely used for the treatment of mitochondrial disorders. Mitochondrial diseases are neuromuscular disorders caused by impairment of the respiratory chain and increased generation of reactive oxygen...
Ufuk Cobanoglu et al.
Asian Pacific journal of cancer prevention : APJCP, 12(6), 1399-1403 (2011-12-01)
Early diagnosis and prevention is very important for lung cancer patients. Previous studies have emphasized that the level of coenzyme Q10 (CoQ10), present primarily in mitochondria, decreases with age and is low in patients with chronic diseases. Our goal was...
Simone Onur et al.
BioFactors (Oxford, England), 41(4), 211-221 (2015-08-01)
Coenzyme Q10 (CoQ10 ) exists in a reduced (ubiquinol) and an oxidized (ubiquinone) form in all human tissues and functions, amongst others, in the respiratory chain, redox-cycles, and gene expression. As the status of CoQ10 is an important risk factor...
Ruolin Li et al.
Autophagy, 1-17 (2021-06-08)
Olfactory dysfunction, one of the earliest non-motor symptoms of Parkinson disease (PD), is accompanied by abnormal deposition of SNCA/α-synuclein in the olfactory bulb (OB). The macroautophagy/autophagy-lysosome pathway (ALP) plays an important role in degrading pathological SNCA and modulating this pathway...
Monika Joanna Turkowicz et al.
BioFactors (Oxford, England), 39(2), 176-185 (2013-01-11)
The article discusses analytical problems related to the determination of coenzyme Q10 in biological samples. The assaying of coenzyme Q10 in complex samples, such as plasma, tissues, or food items requires meticulous sample preparation prior to final quantification. The process...
Mette Lundgren Nielsen et al.
Ugeskrift for laeger, 173(46), 2943-2948 (2011-11-19)
Statin treatment can cause muscular side effects. It has been suggested that the mechanism is reduced synthesis of coenzyme Q10 (coQ10) and a subsequent dysfunction of the respiratory chain. A literature review resulted in insufficient evidence supporting this theory. It...
Ke Li et al.
Biomedical chromatography : BMC, 20(10), 1082-1086 (2006-04-04)
A high-performance liquid chromatographic (HPLC) method for the analysis of coenzyme Q10 (CoQ10) in human seminal plasma was developed and applied to investigate its clinical significance as a reference index relating to oxidative stress and infertile status of spermatozoa. After...
Keren K Griffiths et al.
FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 34(6), 7404-7426 (2020-04-21)
Fragile X syndrome (FXS) is the leading known inherited intellectual disability and the most common genetic cause of autism. The full mutation results in transcriptional silencing of the Fmr1 gene and loss of fragile X mental retardation protein (FMRP) expression....
Seema R Lalani et al.
Archives of neurology, 62(2), 317-320 (2005-02-16)
Primary coenzyme Q(10) (CoQ(10)) deficiency is rare. The encephalomyopathic form, described in few families, is characterized by exercise intolerance, recurrent myoglobinuria, developmental delay, ataxia, and seizures. To report a rare manifestation of CoQ(10) deficiency with isolated mitochondrial myopathy without central...
K Folkers et al.
Biochemical and biophysical research communications, 176(2), 786-791 (1991-05-10)
Coenzyme Q10 (CoQ10) is indispensable to biochemical mechanisms of bioenergetics, and it has a non-specific role as an antioxidant. CoQ10 has shown a hematological activity for the human and has shown an influence on the host defense system. The T4/T8...
Shirin Kalyan et al.
The Journal of clinical endocrinology and metabolism, 99(4), 1307-1313 (2014-01-16)
Nitrogen-bisphosphonates (N-BPs) are the most widely used drugs for bone fragility disorders. Long-term or high-dose N-BP use is associated with unusual serious side effects such as osteonecrosis of the jaw, musculoskeletal pain, and atypical fractures of long bones. It has...
G Siciliano et al.
Brain research bulletin, 56(3-4), 405-410 (2001-11-24)
Steinert's myotonic dystrophy (DM) is a genetic autosomal dominant disease and the most frequent muscular dystrophy in adulthood. Although causative mutation is recognized as a CTG trinucleotide expansion on 19q13.3, pathogenic mechanisms of multisystem involvement of DM are still under...
David L Gasser et al.
American journal of physiology. Renal physiology, 305(8), F1228-F1238 (2013-08-09)
Focal segmental glomerulosclerosis (FSGS) and collapsing glomerulopathy are common causes of nephrotic syndrome. Variants in >20 genes, including genes critical for mitochondrial function, have been associated with these podocyte diseases. One such gene, PDSS2, is required for synthesis of the...
Michelle Grace Acoba et al.
Cell reports, 34(11), 108869-108869 (2021-03-18)
Mitochondrial carriers (MCs) mediate the passage of small molecules across the inner mitochondrial membrane (IMM), enabling regulated crosstalk between compartmentalized reactions. Despite MCs representing the largest family of solute carriers in mammals, most have not been subjected to a comprehensive...
C Q Ye et al.
BioFactors (Oxford, England), 1(4), 303-306 (1988-12-01)
Two situations required a modified determination of coenzyme Q10 (CoQ10) in human blood and organ tissue. Blood from patients with AIDS and cancer raised apprehensions about safety to an analyst, and the number of specimens for analysis is increasing enormously....
Mohammad Abul Kaisar et al.
Brain research, 1627, 90-100 (2015-09-28)
Blood Brain Barrier (BBB) exposed to realistic concentrations (comparable to a chronic heavy smoker) of Cigarette Smoke Extract (CSE) triggers a strong endothelial inflammatory response which can lead to the onset of neurological disorders. The involvement of Reactive Oxygen Species...
Herbert G Markley
Headache, 52 Suppl 2, 81-87 (2012-10-17)
We conducted a short review of relevant literature which contends that migraine is associated with a wide-spread metabolic abnormality of mitochondrial oxidative metabolism, leading to the use of riboflavin and coenzyme Q10 as prophylactic therapy for migraine. Riboflavin and coenzyme...
S Zierz et al.
Journal of neurology, 236(2), 97-101 (1989-02-01)
Coenzyme Q10 (CoQ) was measured in serum and muscle of 17 patients with ophthalmoplegia plus (including 5 patients with Kearns-Sayre syndrome), in muscle of 9 patients with neurogenic atrophies, 5 patients with myositis, and 5 patients with progressive muscular dystrophies...
Iulia Stratulat et al.
Food chemistry, 141(3), 2707-2712 (2013-07-23)
Coenzyme Q10 (CoQ10) was encapsulated successfully in a nutraceutical formulation composed of calcium caseinate, flaxseed oil and lecithin. The effect of CoQ10 on the physico-chemical stability of emulsions was compared to emulsions without CoQ10. According to ATR-FTIR analysis, emulsions were...
William D Shrader et al.
Bioorganic & medicinal chemistry letters, 21(12), 3693-3698 (2011-05-24)
We report that α-tocotrienol quinone (ATQ3) is a metabolite of α-tocotrienol, and that ATQ3 is a potent cellular protectant against oxidative stress and aging. ATQ3 is orally bioavailable, crosses the blood-brain barrier, and has demonstrated clinical response in inherited mitochondrial...
Miyuki Nishijima et al.
International journal of systematic and evolutionary microbiology, 63(Pt 11), 3981-3988 (2013-06-25)
Ten strains of Gram-stain-negative, rod-shaped, non-spore-forming bacteria were isolated from the burial mound soil collected before the dismantling and samples collected during the dismantling work on the Takamatsuzuka Tumulus in Asuka village, Nara Prefecture, Japan in 2007. On the basis...
Valentina Emmanuele et al.
Archives of neurology, 69(8), 978-983 (2012-04-12)
Coenzyme Q(10) (CoQ(10)) deficiency has been associated with 5 major clinical phenotypes: encephalomyopathy, severe infantile multisystemic disease, nephropathy, cerebellar ataxia, and isolated myopathy. Primary CoQ(10) deficiency is due to defects in CoQ(10) biosynthesis, while secondary forms are due to other...
Sarah Nicolai et al.
Science immunology, 5(43) (2020-01-05)
During industrialization, humans have been exposed to increasing numbers of foreign chemicals. Failure of the immune system to tolerate drugs, cosmetics, and other skin products causes allergic contact dermatitis, a T cell-mediated disease with rising prevalence. Models of αβ T...
Steven Lunetta et al.
Journal of AOAC International, 91(4), 702-708 (2008-08-30)
An international collaborative study was conducted of a high-performance liquid chromatographic (HPLC)-UV method for the determination of coenzyme Q10 (CoQ10, ubidecarenone) in raw materials and dietary supplements. Ten collaborating laboratories determined the total CoQ10 content in 8 blind duplicate samples....
H H Szeto et al.
Clinical pharmacology and therapeutics, 96(6), 629-633 (2014-11-18)
Mitochondrial medicine is an evolving discipline whose importance derives from the central function of mitochondria in adenosine triphosphate (ATP) production, generation of reactive oxygen species, and cell death by necrosis or apoptosis. Consequently, mitochondrial dysfunction plays an important role in...
Using coenzyme Q10 in clinical practice.
Emily A Brandmeyer et al.
Nursing, 44(3), 63-66 (2014-02-18)
Jia Liu et al.
The Cochrane database of systematic reviews, (12)(12), CD008150-CD008150 (2011-12-14)
A number of preclinical studies in both in vitro and in vivo models of Parkinson's disease have demonstrated that coenzyme Q10 can protect the nigrostriatal dopaminergic system. Some clinical trials have looked at the neuroprotective effects of coenzyme Q10 in...
Akalabya Bissoyi et al.
Cryo letters, 34(5), 453-465 (2014-01-23)
The present work investigates the effects of a variety of natural cryoprotectants in combination on post-thaw viability and apoptosis of cryopreserved mononuclear cells (MNCs) derived from umbilical cord blood. The extracellular cryoprotectants (10 mM) namely trehalose, hydroxyl ethyl starch, polyvinyl...
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