Human cytochrome oxidase deficiencyROBINSON, Brian H.Pediatric research. 2000, Vol 48, Num 5, pp 581-585, issn 0031-3998Article

Acute Tubular Dysfunction With Fanconi Syndrome : A New Manifestation of Mitochondrial CytopathiesDEBRAY, Francois-Guillaume; MEROUANI, Aicha; LAMBERT, Marie et al.American journal of kidney diseases. 2008, Vol 51, Num 4, pp 691-696, issn 0272-6386, 6 p.Article

Removal and recovery of palladium(II) ions from water using micellar-enhanced ultrafiltration with a cationic surfactantGHEZZI, Lisa; ROBINSON, Brian H; SECCO, Fernando et al.Colloids and surfaces. A, Physicochemical and engineering aspects. 2008, Vol 329, Num 1-2, pp 12-17, issn 0927-7757, 6 p.Article

A genomewide linkage-disequilibrium scan localizes the Saguenay-Lac-Saint-Jean cytochrome oxidase deficiency to 2p16LEE, Nana; DALY, Mark J; DELMONTE, Terrye et al.American journal of human genetics. 2001, Vol 68, Num 2, pp 397-409, issn 0002-9297Article

Late onset leigh syndrome and ataxia due to a T to C mutation at bp 9,185 of mitochondrial DNACASTAGNA, Avril E; ADDIS, Jane; MCINNES, Roderick R et al.American journal of medical genetics. Part A. 2007, Vol 143, Num 8, pp 808-816, issn 1552-4825, 9 p.Article

Long-term outcome and clinical spectrum of 73 pediatric patients with mitochondrial diseasesDEBRAY, Francois-Guillaume; LAMBERT, Marie; CHEVALIER, Isabelle et al.Pediatrics (Evanston). 2007, Vol 119, Num 4, pp 722-733, issn 0031-4005, 12 p.Article

Novel mutations in dihydrolipoamide dehydrogenase deficiency in two cousins with borderline-normal PDH complex activityCAMERON, Jessie M; LEVANDOVSKIY, Valeriy; MACKAY, Neviana et al.American journal of medical genetics. Part A. 2006, Vol 140, Num 14, pp 1542-1552, issn 1552-4825, 11 p.Article

Automated spectrophotometric analysis of mitochondrial respiratory chain complex enzyme activities in cultured skin fibroblastsKRAMER, Karen A; OGLESBEE, Devin; SI HOUN HAHN et al.Clinical chemistry (Baltimore, Md.). 2005, Vol 51, Num 11, pp 2110-2116, issn 0009-9147, 7 p.Article

Synthesis, physiochemical characterization, and biocompatibility of a chitosan/dextran-based hydrogel for postsurgical adhesion preventionCABRAL, Jaydee D; ROXBURGH, Marina; ROBINSON, Brian H et al.Journal of materials science. Materials in medicine. 2014, Vol 25, Num 12, pp 2743-2756, issn 0957-4530, 14 p.Article

Preparation and graft copolymerisation of thiolated β-chitin and chitosan derivativesMUNRO, Natasha H; HANTON, Lyall R; MORATTI, Stephen C et al.Carbohydrate polymers. 2009, Vol 78, Num 1, pp 137-145, issn 0144-8617, 9 p.Article

Rolandic Mitochondrial Encephalomyelopathy and MT-ND3 MutationsWERNER, Klaus G. E; MOREL, Chantal F; FEIGENBAUM, Annette S. J et al.Pediatric neurology. 2009, Vol 41, Num 1, pp 27-33, issn 0887-8994, 7 p.Article

Effects of a Novel Chitosan Gel on Mucosal Wound Healing Following Endoscopic Sinus Surgery in a Sheep Model of Chronic RhinosinusitisATHANASIADIS, Theo; BEULE, Achim G; ROBINSON, Brian H et al.The Laryngoscope. 2008, Vol 118, Num 6, pp 1088-1094, issn 0023-852X, 7 p.Article

LRPPRC mutations cause a phenotypically distinct form of Leigh syndrome with cytochrome c oxidase deficiencyDEBRAY, François-Guillaume; MORIN, Charles; ROBINSON, Brian H et al.Journal of medical genetics. 2011, Vol 48, Num 3, pp 183-189, issn 0022-2593, 7 p.Article

Intermittent peripheral weakness as the presenting feature of pyruvate dehydrogenase deficiencyDEBRAY, Francois-G; LAMBERT, Marie; VANASSE, Michel et al.European journal of pediatrics. 2006, Vol 165, Num 7, pp 462-466, issn 0340-6199, 5 p.Article

Pyruvate dehydrogenase phosphatase deficiency : Identification of the first mutation in two brothers and restoration of activity by protein complementationMAJ, Mary C; MACKAY, Neviana; LEVANDOVSKIY, Valeriy et al.The Journal of clinical endocrinology and metabolism. 2005, Vol 90, Num 7, pp 4101-4107, issn 0021-972X, 7 p.Article

Deficiency of pyruvate dehydrogenase caused by novel and known mutations in the E₁α subunitCAMERON, Jessie M; LEVANDOVSKIY, Valeriy; MACKAY, Neviana et al.American journal of medical genetics. 2004, Vol 131A, Num 1, pp 59-66, issn 0148-7299, 8 p.Article

The effect of short-term dimethylglycine treatment on oxygen consumption in cytochrome oxidase deficiency: A double-blind randomized crossover clinical trialLIET, Jean-Michel; PELLETIER, Véronique; ROBINSON, Brian H et al.The Journal of pediatrics. 2003, Vol 142, Num 1, pp 62-66, issn 0022-3476, 5 p.Article

Impairment of pyruvate dehydrogenase activity by acetaldehydeHARD, Marjie L; RAHA, Sandeep; SPINO, Michael et al.Alcohol (Fayetteville, NY). 2001, Vol 25, Num 1, pp 1-8, issn 0741-8329Article

Effect of thyroid hormone on mitochondrial properties and oxidative stress in cells from patients with mtDNA defectsMENZIES, Keir J; ROBINSON, Brian H; HOOD, David A et al.American journal of physiology. Cell physiology. 2009, Vol 65, Num 2, issn 0363-6143, C355-C362Article

Binding of Pd(II) to Pada in water/micellar system : Complex formation, kinetics in water and DTAC solutionGHEZZI, Lisa; ROBINSON, Brian H; SECCO, Fernando et al.Colloids and surfaces. A, Physicochemical and engineering aspects. 2007, Vol 292, Num 2-3, pp 139-147, issn 0927-7757, 9 p.Article

Novel mitochondrial DNA mutations associated with myopathy, cardiomyopathy, renal failure, and deafnessFEIGENBAUM, Annette; BAI, Ren-Kui; DOHERTY, Emily S et al.American journal of medical genetics. Part A. 2006, Vol 140, Num 20, pp 2216-2222, issn 1552-4825, 7 p.Article

Compensatory responses of protein import and transcription factor expression in mitochondrial DNA defectsJOSEPH, Anna-Maria; RUNGI, Arne A; ROBINSON, Brian H et al.American journal of physiology. Cell physiology. 2004, Vol 55, Num 4, pp C867-C875, issn 0363-6143Article

A novel syndrome affecting multiple mitochondrial functions, located by microcell-mediated transfer to chromosome 2p14-2p13SEYDA, Agnieszka; NEWBOLD, Robert F; ROBINSON, Brian H et al.American journal of human genetics. 2001, Vol 68, Num 2, pp 386-396, issn 0002-9297Article

Mitochondria, oxygen free radicals, and apoptosisRAHA, Sandeep; ROBINSON, Brian H.American journal of medical genetics. 2001, Vol 106, Num 1, pp 62-70, issn 0148-7299Article

Variant non-ketotic hyperglycinemia is caused by mutations in LIAS, BOLA3 and the novel gene GLRX5BAKER, Peter R; FRIEDERICH, Marisa W; LEE, Wang-Tso et al.Brain. 2014, Vol 137, pp 366-379, issn 0006-8950, 14 p., 2Article