about
Acyl-CoA dehydrogenase 9 is required for the biogenesis of oxidative phosphorylation complex ICytosolic signaling protein Ecsit also localizes to mitochondria where it interacts with chaperone NDUFAF1 and functions in complex I assemblyDecreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblastsMutations in NDUFAF3 (C3ORF60), encoding an NDUFAF4 (C6ORF66)-interacting complex I assembly protein, cause fatal neonatal mitochondrial diseaseIdentification of mitochondrial complex I assembly intermediates by tracing tagged NDUFS3 demonstrates the entry point of mitochondrial subunitsHuman Golgi antiapoptotic protein modulates intracellular calcium fluxesAutomated quantification and integrative analysis of 2D and 3D mitochondrial shape and network propertiesPhoto-induction and automated quantification of reversible mitochondrial permeability transition pore opening in primary mouse myotubesFunctional expression of mutations in the human NaCl cotransporter: evidence for impaired routing mechanisms in Gitelman's syndromeCytoskeletal restraints regulate homotypic ALCAM-mediated adhesion through PKCalpha independently of Rho-like GTPasesNDUFA2 complex I mutation leads to Leigh diseaseThe non-gastric H,K-ATPase is oligomycin-sensitive and can function as an H+,NH4(+)-ATPaseHomomultimerization of the coxsackievirus 2B protein in living cells visualized by fluorescence resonance energy transfer microscopyPhenotypic knock out of heparan sulfates in myotubes impairs excitation-induced calcium spiking.Modulation of oxidative phosphorylation and redox homeostasis in mitochondrial NDUFS4 deficiency via mesenchymal stem cells.The structural unit of the thiazide-sensitive NaCl cotransporter is a homodimer.Human mitochondrial complex I assembly is mediated by NDUFAF1.mTOR- and HIF-1α-mediated aerobic glycolysis as metabolic basis for trained immunity.Life cell quantification of mitochondrial membrane potential at the single organelle level.Solute diffusion is hindered in the mitochondrial matrixProtein kinase C-mediated inhibition of transmembrane signalling through CCK(A) and CCK(B) receptorsMutational analysis of the potential phosphorylation sites for protein kinase C on the CCK(A) receptorInteractions between mitochondrial reactive oxygen species and cellular glucose metabolism.Mitochondrial ADP/ATP exchange inhibition: a novel off-target mechanism underlying ibipinabant-induced myotoxicityMultiplexed high-content analysis of mitochondrial morphofunction using live-cell microscopy.Sustained accumulation of prelamin A and depletion of lamin A/C both cause oxidative stress and mitochondrial dysfunction but induce different cell fatesTargeting mitochondrial complex I using BAY 87-2243 reduces melanoma tumor growth.Subunit-specific incorporation efficiency and kinetics in mitochondrial complex I homeostasisIncreased mitochondrial ATP production capacity in brain of healthy mice and a mouse model of isolated complex I deficiency after isoflurane anesthesia.OXPHOS mutations and neurodegenerationHuman NADH:ubiquinone oxidoreductase deficiency: radical changes in mitochondrial morphology?Mitochondrial Ca2+ homeostasis in human NADH:ubiquinone oxidoreductase deficiency.Mitochondrial complex I deficiency: from organelle dysfunction to clinical disease.Mitochondrial dynamics in human NADH:ubiquinone oxidoreductase deficiency.Mammalian mitochondrial complex I: biogenesis, regulation, and reactive oxygen species generation.Mouse models for nuclear DNA-encoded mitochondrial complex I deficiency.Complex I disorders: causes, mechanisms, and development of treatment strategies at the cellular level.Pharmacological targeting of mitochondrial complex I deficiency: the cellular level and beyond.Cellular and animal models for mitochondrial complex I deficiency: a focus on the NDUFS4 subunit.Mitochondrial diseases: Drosophila melanogaster as a model to evaluate potential therapeutics.
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P50
description
onderzoeker
@nl
researcher
@en
ricercatore
@it
հետազոտող
@hy
name
Peter H Willems
@en
Peter H Willems
@es
Peter H Willems
@nl
Peter H Willems
@sl
type
label
Peter H Willems
@en
Peter H Willems
@es
Peter H Willems
@nl
Peter H Willems
@sl
altLabel
Peter H.G.M. Willems
@en
prefLabel
Peter H Willems
@en
Peter H Willems
@es
Peter H Willems
@nl
Peter H Willems
@sl
P1053
L-4759-2015
P106
P21
P214
3559149719109711130007
P31
P496
0000-0002-0915-1599