Nephrotic syndrome associated with coenzyme Q10 deficiency due to coenzyme Q2 gene mutation: a case report / 中华肾脏病杂志

The paper reports a case of coenzyme Q10 deficiency nephrotic syndrome associated with coenzyme Q2 gene mutation and reviews the literature on this topic. The patient presented with hematuria, proteinuria, and a diminution of vision as clinical manifestations. But the proteinuria was not relieved after sufficient doses of glucocorticoids for over 2 months. The patient′s birth history was unremarkable, and his parents were both healthy and not consanguineous. Whole exome sequencing revealed that the patient had a mutation of coenzyme Q2 gene at c.973A>G(p.T325A) and c.517C>T(p.R173C). Combined with renal biopsy pathology, the patient was diagnosed with hereditary nephropathy and started the supplements of coenzyme Q10 after stopping glucocorticoid treatments immediately. After 5 weeks of therapy, the patient′s 24-h urine protein quantification decreased from 6.01 g to 1.53 g.

Avaliação dos efeitos da coenzima Q10 utilizada como coadjuvante local e/ou sistêmica ao tratamento mecânico da periodontite experimental em ratos tratados com nicotina / Evaluation of the effects of coenzyme Q10 as local and/or systemic adjuvant to mechanical treatment of experimental periodontitis in rats treated ith nicotine

O propósito do estudo foi avaliar a efetividade da raspagem e alisamento radicular (RAR) associado à coenzima Q10 (CQ10) administrada localmente e/ou sistemicamente no tratamento da periodontite experimental (PE) em ratos tratados sistemicamente com nicotina (NIC). 128 ratos (Wistar) foram divididos em oito grupos (n=16). Durante todo o período experimental, os animais receberam duas injeções subcutâneas diárias de 3mg/kg de hemissulfato de nicotina ou solução salina (SS) na região dorsal, com 12 horas de intervalo entre elas, começando nos 30 dias que antecederam à indução da PE. Após 15 dias da indução da PE, o protocolo de RAR foi realizado bem como o tratamento coadjuvante local e/ou sistêmico com CQ10, com e sem tratamento com a NIC, sendo: SS-PE-RAR e NIC-PE-RAR: irrigação subgengival com SS; SS-PE-RAR/Q10L e NIC-PE-RAR/Q10L: irrigação subgengival com 1ml solução de CQ10; SS-PE-RAR/Q10S e NIC-PE-RAR/Q10S: gavagem gástrica diária com 120 mg de CQ10; SS-PE-RAR/Q10LS e NIC-PE-RAR/Q10LS: irrigação subgengival com 1ml solução de CQ10 e gavagem gástrica diária com 120 mg de CQ10. As eutanásias foram realizadas 7 e 28 dias após tratamento. As peças coletadas foram processadas com desmineralização para as análises histopatológica, histométrica e imunoistoquímica para detecção de TRAP. Os dados foram submetidos ao teste paramétrico Anova two-way e pós-teste de Tukey. O nível de significância adotado foi de 5% (p≤0,05). Na análise histopatológica, pode-se observar que os grupos NIC-PE-RAR-Q10L E NIC-PE-RAR-Q10LS apresentaram tecidos periodontais com aspecto de normalidade, com preservação da inserção conjuntiva e de região de furca preservada aos 7 e 28 dias, de modo distinto do grupo NIC-PE-RAR e NIC-PE-RAR-Q10S em ambos os períodos. Na análise histométrica, pode-se observar maior porcentagem de osso na furca (POF) (p≤0,05) nos grupos NIC-PE-RAR-Q10L, NIC-PE-RAR-Q10S e NIC-PERAR-Q10LS em comparação com o grupo NIC-PE-RAR em ambos os períodos e também com o grupo SS-PE-RAR aos 28 dias. Pode-se observar menor número de células TRAP positivas (p≤0,05) no grupo NIC-PE-RAR-Q10L quando comparado aos grupos SS-PE-RAR E NIC-PE-RAR aos 7 dias e no grupo NIC-PE-RAR-Q10LS quando comparado aos mesmos grupos aos 28 dias. Conclui-se que RAR associado à CQ10 utilizada local e local/sistemicamente no tratamento da PE em ratos tratados sistemicamente com nicotina foram efetivas mostrando resultados favoráveis nas análises histopatológica, histométrica e imunoistoquímica(AU)

The aim of this study was to evaluate the effectiveness of scaling and root planing (SRP) combined with adjunctive local and/or systemic administration of coenzyme Q10 (CQ10) for the treatment of experimental periodontitis (EP) in rats systemically treated with nicotine (NIC). 128 Wistar rats were divided into 8 groups (n=16). Throughout the experiment, animals received two subcutaneous injections of either 3mg/kg nicotine hemissulfate or physiological saline solution (PSS) with 12 h interval between them. These injections were initiated 30 days prior EP induction. 15 days after EP induction, the protocol for SRP was performed together (or not) with local and/or systemic adjunctive CQ10 administration in animals treat with either NIC or PSS, as described: PSS-EP-SRP and NIC-EP-SRP: subgingival irrigation with PSS; PSS-EP-SRP/Q10L and NIC-EP-SRP/Q10L: subgingival irrigation with 1ml of CQ10 solution; PSS-EP-SRP/Q10S and NIC-EP-SRP/Q10S: daily gastric gavage with 120 mg of CQ10; PSS-EP-SRP/Q10LS and NIC-EP-SRP/Q10LS: subgingival irrigation with 1ml of CQ10 solution and daily gastric gavage with 120 mg of CQ10. The euthanasia was performed at 7 and 28 days after treatment. The specimens were collected and processed for histopathologic, histometric and immunochemical for of TRAP analyzes. The data were submitted to the two-way ANOVA and Tukey's post-test. The level of significance adopted was 5% (p≤0.05). In the histopathological analysis, it can be observed that the NIC-PE-RAR-Q10L and NIC-PE-RAR-Q10LS groups presented periodontal tissues with normal aspect, preserving the conjunctival insertion and furca region preserved at 7 and 28 days, differently from the NIC-PE-RAR and NIC-PE-RAR-Q10S groups in both periods. In histometric analysis, a higher percentage of bone in furca (PBF) (p≤0.05) can be observed in the NIC-PE-RAR-Q10L, NIC-PE-RAR-Q10S and NIC-PE-RAR-Q10LS groups compared to the NIC-PE-RAR group in both periods and also with the SS-PE-RAR group at 28 days. A lower number of TRAPpositive cells (p≤0.05) can be observed in the NIC-PE-RAR-Q10L group when compared to the SS-PE-RAR and NIC-PE-RAR groups at 7 days and in the NIC-PE-RAR-Q10LS group when compared to the same groups at 28 days. It was concluded that RAR associated with CQ10 used locally and locally/systemically in the treatment of EP in rats treated systemically with NIC were effective, showing favorable results in histopathological, histometric and immunohistochemical analyses(AU)

Risk Assessment of "Other Substances" – Coenzyme Q10

The Norwegian Scientific Committee for Food Safety (Vitenskapskomiteen for mattrygghet, VKM) has, at the request of the Norwegian Food Safety Authority (Mattilsynet; NFSA), assessed the risk of "other substances" in food supplements and energy drinks sold in Norway. VKM has assessed the risk of doses given by NFSA. These risk assessments will provide NFSA with the scientific basis while regulating the addition of “other substances” to food supplements and other foods. "Other substances" are described in the food supplement directive 2002/46/EC as substances other than vitamins or minerals t hat have a nutritional and/or physiological effect. It is added mainly to food supplements, but also to energy drinks and other foods. In this series of risk assessments of "other substances", VKM has not evaluated any potential beneficial effects from these substances, only possible adverse effects. The present risk assessment of coenzyme Q10 (CoQ10) is based on previous risk assessments and articles retrieved from a literature search. According to information from NFSA, CoQ10 is an ingredient in food supplements sold in Norway. NFSA has requested a risk assessment of intake of 100 mg/day of CoQ10 in food supplements. CoQ10 (CAS no. 303-98-0) is a naturally-occurring, lipid-soluble compound present in all tissues in humans. Ubiquinone is the totally oxidized form (CoQ10), whereas ubiquinol (CoQ10H2) is the totally reduced form. Meat and fish are the food sources richest in CoQ10. CoQ10 intake from the diet ranges between 3 and 6 mg/day in developed countries. The total body pool of CoQ10 is estimated to be approximately 0.5–1.5 g in an adult. Several studies of CoQ10 (both oxidized and reduced form) have been performed in healthy humans (adults) and animals, showing fairly similar results. The adverse effects reported in a small number of human subjects were generally limited to mild gastrointestinal symptoms such as nausea and stomach upset. In humans, orally ingested CoQ10 was well tolerated at doses up to 900 mg/day (corresponding to 12.9 mg/kg bw per day in a 70 kg adult) over periods up to one month. With regard to animal studies, the lack of adverse effects of CoQ10 doses up to 1200 mg/kg per day in long-term toxicity studies supported and extended the results from the human studies. No studies on children (10 to <14 years) and adolescents (14 to <18 years) were identified. Based on the included literature there was no evidence indicating that age affects tolerance for CoQ10. Therefore, in this risk characterisation the same tolerance as for adults was assumed for these age groups (adjusted for body weight). From a daily dose of 100 mg CoQ10, the daily exposure is 2.3 mg/kg bw for children (10 to <14 years), 1.6 mg/kg bw for adolescents (14 to <18 years), and 1.4 mg/kg bw for adults (≥18 years). For the risk characterization, the values used for comparison with the estimated exposure are 900 mg/day (corresponding to 12.9 mg/kg bw per day in a 70 kg adult) based on human studies (4 weeks) and the no observed adverse effect level (NOAEL) of 1200 mg/kg bw per day based on a long-term toxicity study in rats (52 weeks). The margin of exposure (MOE) approach is used for the rat study; that is the ratio of the NOAEL to the exposure. An acceptable MOE value for a NOAEL-based assessment of CoQ10 based on an animal study is ≥100, which includes a factor 10 for extrapolation from animals to humans, and a factor 10 for interindividual human variation. Comparing the NOAEL from a long-term toxicity study in rats with the estimated exposure for the different age groups, it is unlikely that a daily dose of 100 mg/day of CoQ10 causes adverse health effects in children above 10 years, adolescents and adults. Comparing the dose reported to be well tolerated for healthy adults directly with the estimated exposure, it is unlikely that a daily dose of 100 mg/day of CoQ10 causes adverse health effects in children above 10 years, adolescents and adults. VKM concludes that it is unlikely that a daily dose of 100 mg of CoQ10 from food supplements causes adverse health effects in children (10 to <14 years), adolescents (14 to <18 years) and adults (≥18 years).

Therapeutic effects of proanthocyanidin and coenzyme Q10 on nitrogen mustard-induced ocular injury / Efeitos terapêuticos da proantocianidina e da coenzima q10 em lesão ocular induzida por mostarda de nitrogênio

ABSTRACT Purpose: Nitrogen mustard (NM) is a devastating casualty agent in chemical warfare. There is no effective antidote to treat NM-induced ocular injury. We aimed to assess the effects of proanthocyanidin (PAC) and coenzyme Q10 (CoQ10) on NM-induced ocular injury. Methods: Eighteen male rats were divided into the following 4 groups: NM, NM + PAC, NM + CoQ10, and control. The 3 NM groups received a single dose of NM (0.02 mg/μL) on the right eye to induce ocular injury. The control group received saline only. Thirty minutes after the application of NM, the NM + PAC group received PAC (100 mg/kg) via gastric gavage, while the NM + CoQ10 group received CoQ10 (10 mg/kg) via intraperitoneal injection. PAC and CoQ10 were administered once a day for 5 consecutive days. The rats were then sacrificed. Macroscopic images of the eyes were examined and eye tissues were collected for histology. Results: The treatment groups were compared to the control group with regard to both corneal opacity and lid injury scores. The findings were not significantly different for both the NM + PAC and NM + CoQ10 groups. In both the NM + PAC and NM + CoQ10 groups, the histological changes seen in the NM group demonstrated improvement. Conclusions: Our results indicate that PAC and CoQ10 treatments have therapeutic effects on NM-induced ocular injury in a rat model. PAC and CoQ10 may be novel options in patients with NM-induced ocular injury.

RESUMO Objetivo: A mostarda de nitrogênio (MN) é um agente de guerra química devastador. Não existe um antídoto eficaz para tratar lesões oculares induzidas por MN. Nosso objetivo foi avaliar os efeitos da proantocianidina (PAC) e da coenzima Q10 (CoQ10) na lesão ocular induzida por MN. Métodos: Dezoito ratos machos foram divididos em 4 grupos: MN, MN + PAC, MN + CoQ10 e Controle. Três grupos receberam uma dose única de MN (0,02 mg/μL) destilada no olho direito para gerar lesão ocular. Os animais do grupo controle receberam apenas solução salina. Trinta minutos após a aplicação de MN nos animais, o grupo MN + PAC recebeu PAC (100 mg/kg) por gavagem gástrica, enquanto a CoQ10 (10 mg/kg) foi administrada ao grupo MN + CoQ10 por meio de injeção intraperitoneal. A administração de PAC e de CoQ10 foi realizada uma vez por dia, durante 5 dias consecutivos. Os ratos foram, então, sacrificados. Imagens macroscópicas dos olhos foram examinadas e tecidos oculares foram coletados para histologia. Resultados: Os grupos de tratamento foram comparados ao grupo de controle quanto à opacidade da córnea e quanto aos escores de lesão da cobertura da córnea. Os resultados foram insignificantes para ambos os grupos. Ambos, o grupo MN+PAC e o grupo MN+CoQ10, apresentaram melhoras das alterações histológicas observadas no grupo MN. Conclusões: Nossos resultados indicam que os tratamentos com PAC e com CoQ10 têm efeitos terapêuticos sobre lesões oculares induzidas por MN em um modelo em ratos. A proantocianidina e a CoQ10 podem ser uma nova opção nesses casos.

A familial case of mitochondrial leukoencephalopathy related to NADH dehydrogenase (ubiquinone)flavoprotein 2 gene mutation / 中华实用儿科临床杂志

Objective To analyze the clinical and imaging features of 2 siblings with leukoencephalopathy due to NADH dehydrogenase (ubiquinone)flavoprotein 2 (NDUFV2) gene mutation,in order to better understand and diagnose it earlier.Methods Clinical and follow-up data of the proband and his brother were collected.Clinical features including symptoms,signs and cranial magnetic resonance imaging (MRI) were analyzed,and 2 patients were followed up for a long time.Sanger sequencing,targeted next generation sequencing,and whole exome sequencing were performed to identify potential genetic variations in the 2 patients and their parents.Results (1) Clinical characteristics and follow-up:ages of onset were 4 months and 1 year respectively.Both of the patients presented rapid motor regression hyperinyotonia,positive pathological character.During the follow-up the condition became stable,motor function and cognition improved gradually after cocktail therapy.(2) Brain MRI of the 2 patients showed prominent abnormalities in deep cerebral white matter,presenting T1 hypointense,T2 and fluid attenuated inversion recovery (FLAIR) hyperintense in the periventricular area.FLAIR images revealed that the abnormal white matter was partially rarefied and cavitated.Diffusion weighted images (DWI) showed high signals along the periphery of the involved areas.The follow-up MRI showed the cavitation still existed and even expanded,and DWI showed regional linear or spotty high signals around the original lesions.(3) Novel mutations in NDUFV2 gene,c.467T>A and c.404G>C,were identified in proband and his brother.The former inherited from his father,while the latter inherited from his mother,which was the new mutation not reported in the international.Conclusions The clinical features of the brothers presented subacute leukoencephalopathy with relatively stable or improved outcome.This was distinctive from the phenotypic features reported in 12 cases with hypertrophic cardiomyopathy or Leigh syndrome.The finding expanded the phenotypic spectrum of NDUFV2 mutations.Pathogenic gene of these patients which is the basis of genetic counseling for this family was determined.

Clinical and genetic characters of 8 Chinese children with ADCK4-associated glomerulopathy / 中华肾脏病杂志

Objective@#To investigate the clinical and genetic character of Chinese children with the aarF domain containing kinase 4 (ADCK4)-associated glomerulopathy.@*Methods@#Applying next generation sequencing to detect possible gene mutation(renal disease associated monogene was pooled as one panel) in 69 children with steroid-resistant nephrotic syndrome (SRNS) or persistent proteinuria of unknown origin. Sanger sequencing was used to confirm the significant mutations found in the children and to validate these mutation sites in their patients. Using online software (PolyPhen2, SIFT, Mutation Taster) to predict whether the detected missense mutations were disease causing or not. Collecting and analyzing clinical data of children with ADCK4-associated glomerulopathy, which included onset age, clinical manifestation, and renal pathology.@*Results@#The ADCK4 gene mutation was detected in 8 children with a positive rate of 11.6% (8 out of 69), among which 3 patients carried homozygous c.748G>C mutation, 3 patients carried homologous c.737G>A mutation, 1 patient carried compound heterozygous mutation(c.748G>C and c.737G>A), and 1 patient carried compound heterologous mutation(c.551A>G and c.737G>A). Collectively, there were only 3 mutation sites found in total 8 patients, in which the mutation sites of c.748G>C and c.737G>A had high detection frequency in these 8 patients. These 3 mutation sites were all missense mutation which were predicted to be disease causing by online software and not reported before. The average onset age was 6.5 years (2 years-11.75 years). Four patients presented with SRNS and the other 4 presented with persistent proteinuria. All 8 patients had no extrarenal manifestation, renal biopsy revealed focal segmental glomerulosclerosis (FSGS) in most patients, among which 3 cases had gone to end-stage renal disease (ESRD) at disease onset, and 2 cases progressed to ESRD 2 and 5 years after onset respectively. Seven patients had received glucocorticoid and/or immunosuppressive drug while only one patient getting partial response. All 8 patients were treated with large amount of coenzyme Q10 (15 mg·kg-1·d-1) after definite diagnosis of ADCK4 mutation-some patients had acquired encouraging curative effect.@*Conclusions@#ADCK4-associated glomerulopathy is not rare especially in the children with SRNS. The onset age is relatively old and the extrarenal manifestation is less common. FSGS is a main pathology type. Patients usually have no response to immunosuppressive therapy, but may benefit from addition of large amount of coenzyme Q10. Some patients may only manifest with insidious proteinuria, causing the early diagnosis to be difficult, which deserves more attention. Three new missense mutations expand disease causing mutation repertoire of ADCK4 gene, among which the two sites of c.748G>C and c.737G>A may be mutation hotspot of ADCK4-associated glomerulopathy in Chinese population, and need further study.

Study of coenzyme Q10 in the liver of preeclampsia pregnant rats / 中华妇产科杂志

Objective To investigate the protective effect of coenzyme Q10 (CoQ10) in the liver of preeclampsiapregnant rats and the potential etiology. Methods Fifty pregnant SD rats were equally divided into the normal pregnant (NP) group (n=10) and the preeclampsia (PE) group (n=40) randomly. The PE rats (n=40) were equally divided into four groups randomly,distilled water(DW)group,CoQ10 group, CoQ10 combined magnesium(CM) group and magnesium (Mg) group were established by treating the preeclampsia rats on day 15 to 21 of gestation with different measures. As for all the 50 rats, systolic blood pressure (SBP) of rat tail was detected on day 10, 15 and 21 of gestation respectively, 24 hours proteinuria analysis were detected on day 10, 15 and 21 of gestation respectively, levels of alanine aminotransferase (ALT), aspartate aminotransferase (AST) in blood andsuperoxide dismutase (SOD), glutathione peroxidase (GSH-PX), malondialdehyde (MDA), caspase-3, Bcl-2 and Bax protein expression in liver tissue were detected by western blot assay on day 21 of gestation. Results (1)SBP and 24 hours proteinuria analysis:there was no statistic difference among all the five groups on day 10 of gestation (P>0.05). Whereas, SBP and 24 hours proteinuria analysis were significantly higher in CoQ10 group, CoQ10 combined CM group, CM group and DW group than that in NP group on day 15, 21 of gestation (P<0.05). And SBP and 24 hours proteinuria analysis were significantly lower in CoQ10 group, CoQ10 combined CM group and CM group than that in DW group on day 21 of gestation (P<0.05). (2) Liver function: among CoQ10 group, CoQ10 combined CM group, CM group, DW group and NP group, serum levels of ALT were respectively(52±7),(34±9),(49±10), (70 ± 19),(30 ± 7)U/L;and serum levels of AST were respectively(169 ± 25),(84 ± 11),(159 ± 20),(281 ± 26)and(78±18)U/L. ALT and AST serum levels were significantly higher in CoQ10 group, CM group and DW group than that in NP group (P<0.05). ALT and AST serum levels were significant lower in CoQ10 combined CM group than those in CoQ10 group, CM group and DW group, respectively (P<0.05). ALT and AST serum levels were significant lower in CoQ10 group and CM group than that in DW group, respectively (P<0.05). (3) SOD, GSH-PX, MDA, caspase-3, Bcl-2 and Bax expression in liver tissue of rats: SOD expression was significant higher in CoQ10 group, CoQ10 combined CM group than thoes in CM group, DW group and NP group(P<0.05);SOD expression was significant lower in CM group, DW grouo than thoes in NP group(P<0.05);and SOD expression was significant higher in CM group than that in DW group(P<0.05). Compared with CoQ10 group, CoQ10 combined CM group, CW group and DW group respectively, the GSH-PX and Bcl-2 protein expressions were significant higher in NP group(P<0.05), while MDA, caspase-3 and Bax protein expressions were significant lower in NP group(P<0.05);compared with CoQ10 group, CoQ10 combined CM group and CW group respectively, the GSH-PX and Bcl-2 protein expressions were significant lower in DW group (P<0.05), while MDA, caspase-3 and Bax protein expressions were significant higher in DW group (P<0.05). Conclusions Oxidative stress and apoptosis levles were upregulated in PE pregnant liver tissues. CoQ10 could effectively protect the liver by improving the liver functions and decreasing the apoptosis of liver cells in PE pregnant rats, and markedly decrease the oxidative stress and apoptosis in the livers. The protective roles of CoQ10 in liver might through its function of anti-oxidative stress and inhibiting cell apoptosis by regulating the balance of Bcl-2/Bax.

Efficacy of coenzyme Q10 in preventing propofol infusion syndrome in rats / 中华麻醉学杂志

Objective To evaluate the efficacy of coenzyme Q10 in preventing propofol infusion syndrome in rats.Methods Thirty pathogen-free healthy male Sprague-Dawley rats,aged 8-10 weeks,weighing 250-280 g,were randomly divided into 3 groups (n=10 each) using a random number table:control group (group C),propofol group (group P) and coenzyme Q10 group (group CoQ10).Normal saline was infused intravenously in group C.In group P,1％ propofol in medium-and long-chain triglyceride emulsion injection was infused intravenously.In group CoQ10,CoQ10 100 mg/kg was administrated by intragastric gavage,and 1 h later propofol was infused intravenously.The infusion rate was 20mg·kg-1 ·h-1 within the first6hand40mg· kg-1 · h-1fortherest6h,and the total time was 12hin the three groups.Immediately after the start of administration (To),and at 6 and 12 h after the start of administration (T1,2),blood samples 2 ml were taken from the common carotid artery,with 0.5 ml for blood gas analysis and 1.5 ml for determination of the levels of serum aspartate aminotransferase (AST),alanine aminotransferase (ALT),creatine kinase (CK),creatine kinase isoenzyme-MB (CK-MB),cardiac troponin Ⅰ (cTnⅠ),blood urea nitrogen (BUN) and creatinine (Cr).After blood sampling,the rats were sacrificed,and myocardial tissues were obtained for pathological examination.Results Compared with group C,the serum AST,ALT,CK,CK-MB and cTnⅠ levels were significantly increased at T1,2 (P＜0.05),no significant changes were found in serum BUN and Cr levels (P＞0.05),the pathological changes of myocardium were aggravated in P and CoQ10 groups.Compared with group P,the serum AST,ALT,CK,CK-MB and cTnⅠ levels were significantly decreased at T1,2 (P＜0.05),no significant changes were found in serum BUN and Cr levels (P＞0.05),and the pathological changes of myocardium were significantly attenuated in group CoQ10.Conclusion Coenzyme Q10 can effectively prevent the development of propofol infusion syndrome in rats.

The role of idehenone in the treatment of nervous system diseases New knowledge and new findings / 国际脑血管病杂志

Idebenone is a synthetic analogue of coenzyme Q10. As a potent antioxidant,idebenone operates under low oxygen tension situations, protects cell membranes and mitochondria from oxidative damage through inhibiting lipid peroxidation, thereby protects against cerebral ischemia and nervous system damage. Idebenone also interacts with the mitochondrial electron transport chain and maintains the formation of ATP under ischemic conditions. Because idebenone has a good tolerability and safety, it is expected as a neuroprotective agent for the treatment of acute ischernic stroke. Studies in recent years have showed that idebenone has a certain therapeutic effect in a variety of nervous system diseases involving mitochondrial dysfunction and oxidative stress damage, such as mitochodrial encephalomyopathy, lactic acidosis, stroke-like episodes syndrome, Friedreich's ataxia, Alzheimer's diseases, Leber's hereditary optic neuropathy, and Duchenne muscular dystrophy. At present, some clinical trials in the mitochondria-related diseases and neuromuscular diseases are underway, and their results are expected to further expand the indications of idebenone.

Progresses of diagnosis and therapy in multiple acyl-CoA dehydrogenase deficiency / 国际儿科学杂志

Multiple acyl-CoA dehydrogenase deficiency (MADD) is a common inborn error of fatty-acid metabolism characterized by vomiting, acidosis and lipid storage myopathy, and the clinical manifestations of MADD are highly heterogeneous. MADD can be diagnosed by the elevation of multiple acyl-carnitine in blood and glutaric acid or other organic acid in urine. The neonatal-onset patients have severe symptoms and poor prognosis. However, oral riboflavin supplementation (can completely rescue) ameliorate the clinical and laboratory disorders rapidly especially to the riboflavin responsive MADD. Additionally, patients not sensitive to riboflavin should also take low lipid, low protein and high carbonhydrate diet besides riboflavin.

Bioavailability of ubiquinone 10 tablets in healthy volunteers / 中国临床药理学与治疗学

Aim To compare the bioavailability of two ubiquinone tablets in healthy volunteers. Methods A HPLC method was used to determine the serum ubiquinone 10 concentrations at 0,1,2,3,4,6,8 and 12 h after oral administration for 7 days ( 20 mg, tid ) in a cross-over test. Results AUC, Cmax and Tpeak of the test tablets were (5.91?1.78)?g?h?ml-1 ,(0.66?0.17)?g?ml-1 and (4.00?1.25) h, respectively,and these of the reference tablets were (6.30?2.09)?g?h?ml-1,(0.70?0.20)?g?ml-1 and (4.60?1.58)h , respectively . All of these parameters between the two kinds of tablets were not significantly different statistically. Conclusion The related bioavailability of the test tablets versus the reference tablets is 93.9%. The two formulations of ubiquinone 10 are bioequivalent.

Chromomycosis Caused by Phialophora Verrucosa: a Case Report, and Ubiquinone System and DNA Sequence Analysis of Pathogen / 中华皮肤科杂志

Objective To report a case of chromomycosis caused by Phialophora verrucosa and explore the laboratory features of the pathogen. Methods Skin lesion was examined by histopathology and fungus culture. The morphology of the isolate was observed by microscopy and scanning electron microscopy. The coenzyme Q system of this isolate was analyzed by HPLC assay. The DNA sequences of LSU rDNA D1/D2 region of this isolate and a standard fungus strain were compared. Results The initial lesion was an erythematous papule that subsequently developed into one or multiple coalescing warty papules or plaques slowly. The bronze-colored spores could were observed in the dermis or macrophages. The isolate grew very slowly, requiring 4 weeks of incubation. Microscopically, no characteristic structures were found on Sabourand′s dextrose agar, while there were vase-like structures, which were referred to as phialides on potato dextrose agar (PDA) and corn meal agar I (CMA-I). The phialides on PDA mostly grew at the top of hypha, but on CMA-I they mostly grew on the side of hypha. The isolate contained coenzyme Q-10, and its DNA sequence of LSU rDNA D1/D2 region completely consistent with those of the standard strain. Conclusion Chromomycosis caused by Phialophora verrucosa is rare in China. It can be diagnosed by fungus culture and histopathological examination. Coenzyme Q system analysis and DNA sequencing can exclude the interference from different phenotypes.

BACTERIAL QUINONE PROFILE FOR THE STUDY OF MICROBIAL COMMUNITY STRUCTURE IN ENVIRONMENTAL SAMPLES / 微生物学通报

Quinone is one of the electron transporters of the microbial respiratory chain. The dominant quinone of one species of bacteria is different from other bacteria. So the quinone profile of environmental samples can reflect the microbial community. This paper briefly introduces the analytical method used for microbial quinones. The microbial community in an activated sludge sample is studied using this method.

Increase in hepatic ubiquinone on administration of diethylhexyl phthalate to the rat.

It is shown for the first time that the content of ubiquinone of liver increases (2·5 fold) on dietary administration of the widely-used industrial plasticizer diethylhexyl phthalate to the rat. The increase is localized almost entirely in mitochondria in which the concentration of the quinone per mg protein is 1·7 times the control. Incorporation of the radioactive precursor (acetate) reveals that the biosynthesis of ubiquinone is increased in the livers of plasticizer-administered animals. The rate of degradation is not altered.