Výsledky vyhledávání - "A. M. F. Martines"

Akademický článek

Personalised modelling of clinical heterogeneity between medium-chain acyl-CoA dehydrogenase patients

Autor: Christoff Odendaal, Emmalie A. Jager, Anne-Claire M. F. Martines, Marcel A. Vieira-Lara, Nicolette C. A. Huijkman, Ligia A. Kiyuna, Albert Gerding, Justina C. Wolters, Rebecca Heiner-Fokkema, Karen van Eunen, Terry G. J. Derks, Barbara M. Bakker

Publikováno v: BMC Biology, Vol 21, Iss 1, Pp 1-22 (2023)

Abstract Background Monogenetic inborn errors of metabolism cause a wide phenotypic heterogeneity that may even differ between family members carrying the same genetic variant. Computational modelling of metabolic networks may identify putative sourc

Externí odkaz: https://doaj.org/article/c317aa533e824a27ae9be54b9fd0b3e9

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Akademický článek

Bistability in fatty-acid oxidation resulting from substrate inhibition.

Autor: Fentaw Abegaz, Anne-Claire M F Martines, Marcel A Vieira-Lara, Melany Rios-Morales, Dirk-Jan Reijngoud, Ernst C Wit, Barbara M Bakker

Publikováno v: PLoS Computational Biology, Vol 17, Iss 8, p e1009259 (2021)

In this study we demonstrated through analytic considerations and numerical studies that the mitochondrial fatty-acid β-oxidation can exhibit bistable-hysteresis behavior. In an experimentally validated computational model we identified a specific r

Externí odkaz: https://doaj.org/article/febc2d2c3e1e4399b08b47a1554814cc

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Akademický článek

The promiscuous enzyme medium-chain 3-keto-acyl-CoA thiolase triggers a vicious cycle in fatty-acid beta-oxidation.

Autor: Anne-Claire M F Martines, Karen van Eunen, Dirk-Jan Reijngoud, Barbara M Bakker

Publikováno v: PLoS Computational Biology, Vol 13, Iss 4, p e1005461 (2017)

Mitochondrial fatty-acid beta-oxidation (mFAO) plays a central role in mammalian energy metabolism. Multiple severe diseases are associated with defects in this pathway. Its kinetic structure is characterized by a complex wiring of which the function

Externí odkaz: https://doaj.org/article/5d0b04246cf74a6e8e156d52acc0ff9f

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Transcriptome analysis suggests a compensatory role of the cofactors coenzyme A and NAD

Autor: Anne-Claire M F, Martines, Albert, Gerding, Sarah, Stolle, Marcel A, Vieira-Lara, Justina C, Wolters, Angelika, Jurdzinski, Laura, Bongiovanni, Alain, de Bruin, Pieter, van der Vlies, Gerben, van der Vries, Vincent W, Bloks, Terry G J, Derks, Dirk-Jan, Reijngoud, Barbara M, Bakker

Publikováno v: Scientific Reports

During fasting, mitochondrial fatty-acid β-oxidation (mFAO) is essential for the generation of glucose by the liver. Children with a loss-of-function deficiency in the mFAO enzyme medium-chain acyl-Coenzyme A dehydrogenase (MCAD) are at serious risk

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::f43adc1cee844f530bd677e64d4c6457
https://pubmed.ncbi.nlm.nih.gov/31601874

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The promiscuous enzyme medium-chain 3-keto-acyl-CoA thiolase triggers a vicious cycle in fatty-acid beta-oxidation

Autor: Karen van Eunen, Barbara M. Bakker, Anne-Claire M F Martines, Dirk-Jan Reijngoud

Publikováno v: PLoS Computational Biology, Vol 13, Iss 4, p e1005461 (2017)
PLoS Computational Biology
PLoS Computational Biology, 13(4):e1005461. PUBLIC LIBRARY SCIENCE

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1bb5ca5aa8dcd6a19239466ae553ebd5
http://europepmc.org/articles/PMC5397069?pdf=render

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Living on the edge : Substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders

Autor: Karen van Eunen, Dirk-Jan Reijngoud, K. E. Niezen-Koning, Albert K. Groen, Willemijn J. van Rijt, Anne Claire M F Martines, Rebecca M. Heiner, Barbara M. Bakker, Terry G J Derks, Hjalmar P. Permentier, Justina C. Wolters, Aycha Bleeker, Albert Gerding, Catharina M L Volker-Touw

Publikováno v: BMC Biology
BMC Biology, 14(1):107. BioMed Central Ltd.
BMC Biology, 14. BioMed Central Ltd.

Background Defects in genes involved in mitochondrial fatty-acid oxidation (mFAO) reduce the ability of patients to cope with metabolic challenges. mFAO enzymes accept multiple substrates of different chain length, leading to molecular competition am

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4b21d66b35c86202977865caec5fa6a4
https://dspace.library.uu.nl/handle/1874/345397

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Iron metabolism in the pathogenesis of iron-induced kidney injury

Autor: Harold Tjalsma, Dorine W. Swinkels, Joost G. J. Hoenderop, A. M. F. Martines, Jack F.M. Wetzels, Rosalinde Masereeuw

Publikováno v: Nature Reviews. Nephrology, 9, 385-98
Nature Reviews. Nephrology, 9, 7, pp. 385-98

Contains fulltext : 118694pos.pdf (Author’s version postprint ) (Open Access) In the past 8 years, there has been renewed interest in the role of iron in both acute kidney injury (AKI) and chronic kidney disease (CKD). In patients with kidney disea

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cb69a45516534dd04f7a8293fab8c562
https://doi.org/10.1038/nrneph.2013.98

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