Anaerobic Threshold as Detected from Ventilatory and Metabolic Exercise Responses in Patients with Mitochondrial Respiratory Chain Defect

Autor: J. M. Bogaard, H. Stam, H. R. Scholte, H. F. M. Busch, A. Versprille

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::05d6b38fe11042690cdde73317ce1dae
https://doi.org/10.1159/000413446

Pearson syndrome and the role of deletion dimers and duplications in the mtDNA

Autor: H. R. Scholte, Roselie Jongbloed, J.P.M. Geraedts, J. B. C. de Klerk, H. J. M. Smeets, J.G. Nijland, I.F.M. de Coo, L.J.A.M. Jacobs, Frits A. Wijburg

Publikováno v: Journal of Inherited Metabolic Disease, 27, 47-55. Springer Netherlands
Journal of inherited metabolic disease, 27(1), 47-55. Springer Netherlands

Pearson syndrome is an often fatal multisystem disease associated with mitochondrial DNA rearrangements. Here we report a patient with a novel mtDNA deletion of 3.4 kb ranging from nucleotides 6097 to 9541 in combination with deletion dimers. The mut

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::692883f32f9545dac93c7433a6b6fdcb
https://pubmed.ncbi.nlm.nih.gov/14970745

Rapid isolation of muscle and heart mitochondria, the lability of oxidative phosphorylation and attempts to stabilize the process in vitro by taurine, carnitine and other compounds

Autor: H R, Scholte, Y, Yu, J D, Ross, I I, Oosterkamp, A M, Boonman, H F, Busch

Publikováno v: Molecular and cellular biochemistry. 174(1-2)

We modified the isolation procedure of muscle and heart mitochondria. In human muscle, this resulted in a 3.4 fold higher yield of better coupled mitochondria in half the isolation time. In a preparation from rat muscle we studied factors that affect

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::c8c27f9a81fde6b379be54f20155c3a6
https://pubmed.ncbi.nlm.nih.gov/9309666

Acute myocardial ischaemia induces cardiac carnitine release in man

Autor: W. J. Remme, G. L. Bartels, H. R. Scholte

Publikováno v: European Heart Journal, 84-90. Oxford University Press
ISSUE=18;STARTPAGE=84;ENDPAGE=90;ISSN=0195-668X;TITLE=European Heart Journal

In animal studies, prolonged periods of ischaemia decrease the cardiac carnitine content. However, whether in humans the heart loses carnitine during short-term ischaemia, and whether this is related to ischaemia-induced cardiac dysfunction, is as ye

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::35dacb6393f3de894fe3c01e52ded4b9
https://pure.eur.nl/en/publications/da237c53-0ef6-4cb0-8a2a-4eacb5158d67

[Pathogenesis and pathophysiology of carnitine deficiency, a predictable risk]

Autor: R, Rodrigues Pereira, H, Przyrembel, H R, Scholte

Publikováno v: Tijdschrift voor kindergeneeskunde. 58(5)

When patients suffer from hepato-encephalopathy, (cardio)myopathy, dystrophy, hypoglycaemia, some metabolic diseases and several other disease states, carnitine deficiency should be considered. In this article a survey is given of the pathophysiology

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=pmid________::3f73f3afa2fc6a6b2f9b73ee9c0d26cc
https://pubmed.ncbi.nlm.nih.gov/2247875