Zobrazeno 1 - 10
of 41
pro vyhledávání: '"S J R Heales"'
Publikováno v:
Brain Research. 1036:1-6
The antioxidant glutathione (GSH) plays an important role in protecting the mitochondrial electron transport chain (ETC) from damage by oxidative stress in astrocytes and neurones. Neurones co-cultured with astrocytes have greater GSH levels, compare
Autor:
S. J. R. Heales, P. Guthrie, S Rahman, J-W. Taanman, John M. Land, James V. Leonard, Iain P. Hargreaves
Publikováno v:
Journal of Inherited Metabolic Disease. 25:7-16
Mitochondrial DNA (mtDNA) depletion syndrome (McKusick 251880) is characterized by a progressive quantitative loss of mtDNA resulting in severe mitochondrial dysfunction. A diagnosis of mtDNA depletion can only be confirmed after Southern blot analys
Publikováno v:
Journal of Neurochemistry. 73:1446-1452
The biosynthesis of the physiological messenger nitric oxide (*NO) in neuronal cells is thought to depend on a glial-derived supply of the *NO synthase substrate arginine. To expand our knowledge of the mechanism responsible for this glial-neuronal i
Autor:
Alan M. Pittman, S. J. R. Heales, R.D.S. Pitceathly, Mary G. Sweeney, Michael G. Hanna, Enrico Bugiardini, Cathy E. Woodward, Olivia V. Poole, H Houlden
Publikováno v:
Neuromuscular Disorders. 27:S23
Autor:
John B. Clark, Foppa P, M. P. Brand, Iain P. Hargreaves, S. J. R. Heales, Juan P. Bolaños, VC Stewart, John M. Land, JE Barker
Publikováno v:
Biochemical Society Transactions. 25:939-943
Publikováno v:
Journal of Inherited Metabolic Disease. 23:352-354
Carnitine palmitoyltransferase II (CPT II) converts acylcarnitine esters to the corresponding acyl-CoA derivatives within mitochondria prior to β-oxidation. CPT II deficiency (McKusick 255110) may present early in life, with patients displaying hypo
Autor:
Josie McConnell, Malgorzata S. Martin-Gronert, Anthea Rowlerson, Lucilla Poston, Iain P. Hargreaves, Denise S. Fernandez-Twinn, Piran Shelley, Susan E. Ozanne, S. J. R. Heales
We recently reported insulin resistance in adult offspring of obese C57BL/6J mice. We have now evaluated whether parameters of skeletal muscle structure and function may play a role in insulin resistance in this model of developmental programming. Ob
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3a3b6b070362288d42865c9e6614fae9
https://europepmc.org/articles/PMC2739782/
https://europepmc.org/articles/PMC2739782/
Publikováno v:
Journal of Inherited Metabolic Disease. 22:221-223
Within the central nervous system (CNS), tetrahydrobiopterin (BH 4 ) is an essential cofactor for the aromatic amino acid monoxygenases. Consequently, inborn errors of BH 4 metabolism result in hyperphenylalaninaemia and impaired dopamine and seroton
Publikováno v:
Journal of inherited metabolic disease. 32(2)
The pathogenesis of mitochondrial disorders has largely focused on the impairment of cellular energy metabolism. However, mitochondrial dysfunction has also been implicated as a factor in the initiation of apoptosis due to the translocation of cytoch
Autor:
S J R Heales, A A J Lam
Publikováno v:
Annals of clinical biochemistry. 44(Pt 4)
Assessment of total neopterin and tetrahydrobioterin (BH4) concentrations in cerebrospinal fluid (CSF) can be used to identify potential disorders of BH4 biosynthesis. In this study, we demonstrate that exposure of CSF to nitric oxide leads to an acc