Search Results - "Koopman, W. J. H"

Phytanic acid impairs mitochondrial respiration through protonophoric action by Komen, J. C, Distelmaier, F, Koopman, W. J. H, Wanders, R. J. A, Smeitink, J, Willems, P. H. M. G

“…Refsum disease is a rare, inherited neurodegenerative disorder characterized by accumulation of the dietary branched-chain fatty acid phytanic acid in plasma…”
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Enteric neurons from Parkinson’s disease patients display ex vivo aberrations in mitochondrial structure by Baumuratov, A. S., Antony, P. M. A., Ostaszewski, M., He, F., Salamanca, L., Antunes, L., Weber, J., Longhino, L., Derkinderen, P., Koopman, W. J. H., Diederich, N. J.

“…Based on autopsy material mitochondrial dysfunction has been proposed being part of the pathophysiological cascade of Parkinson’s disease (PD). However, in…”
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Impaired primary mouse myotube formation on crosslinked type I collagen films is enhanced by laminin and entactin by Grefte, S., Adjobo-Hermans, M.J.W., Versteeg, E.M.M., Koopman, W.J.H., Daamen, W.F.

“…[Display omitted] In skeletal muscle, the stem cell niche is important for controlling the quiescent, proliferation and differentiation states of satellite…”
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Transcriptional changes in OXPHOS complex I deficiency are related to anti-oxidant pathways and could explain the disturbed calcium homeostasis by Voets, A.M., Huigsloot, M., Lindsey, P.J., Leenders, A.M., Koopman, W.J.H., Willems, P.H.G.M., Rodenburg, R.J., Smeitink, J.A.M., Smeets, H.J.M.

“…Defective complex I (CI) is the most common type of oxidative phosphorylation disease, with an incidence of 1 in 5000 live births. Here, whole genome…”
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Patient-derived fibroblasts indicate oxidative stress status and may justify antioxidant therapy in OXPHOS disorders by Voets, A.M., Lindsey, P.J., Vanherle, S.J., Timmer, E.D., Esseling, J.J., Koopman, W.J.H., Willems, P.H.G.M., Schoonderwoerd, G.C., De Groote, D., Poll-The, B.T., de Coo, I.F.M., Smeets, H.J.M.

“…Oxidative phosphorylation disorders are often associated with increased oxidative stress and antioxidant therapy is frequently given as treatment. However, the…”
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A guide to diagnosis and treatment of Leigh syndrome by Baertling, Fabian, Rodenburg, Richard J, Schaper, Jörg, Smeitink, Jan A, Koopman, Werner J H, Mayatepek, Ertan, Morava, Eva, Distelmaier, Felix

“…Leigh syndrome is a devastating neurodegenerative disease, typically manifesting in infancy or early childhood. However, also late-onset cases have been…”
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