348 results on '"Taanman, Jan-Willem"'
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2. Prostacyclin mimetics inhibit DRP1-mediated pro-proliferative mitochondrial fragmentation in pulmonary arterial hypertension
3. WED-099-YI Hyperammonemia-induced mitochondrial dysfunction is abrogated by targeting toll-like receptor 4
4. Deoxyguanosine kinase deficiency: natural history and liver transplant outcome
5. Huntington’s disease affects mitochondrial network dynamics predisposing to pathogenic mitochondrial DNA mutations
6. Sirtuin 5 depletion impairs mitochondrial function in human proximal tubular epithelial cells
7. Mito and Glycolysis Stress Tests for Enteroendocrine Cells - Hutu-80 v1
8. Remodelling of the Mitochondrial Bioenergetic Pathways in Human Cultured Fibroblasts with Carbohydrates
9. Creation of an open-access, mutation-defined fibroblast resource for neurological disease research.
10. Mitochondria as target to inhibit proliferation and induce apoptosis of cancer cells: the effects of doxycycline and gemcitabine
11. Contributors
12. Mitochondrial DNA: Structure, Genetics, Replication and Defects
13. Exploring metabolism in scleroderma reveals opportunities for pharmacological intervention for therapy in fibrosis
14. Mutant TRIAP1 causes impaired mitochondrial bioenergetics and myopathy
15. Transcriptomic analysis confirms that PNPLA3 I148M variant is associated with impaired mitochondrial function and antioxidant response in 3D cultured human hepatic stellate cells and liver tissue of patients with NAFLD
16. Loss of PINK1 or Parkin Function Results in a Progressive Loss of Mitochondrial Function
17. Contributors
18. Mitochondrial cristae remodelling is associated with disrupted OPA1 oligomerisation in the Huntingtonʼs disease R6/2 fragment model
19. Correction: The clinical spectrum and natural history of early-onset diseases due to DNA polymerase gamma mutations
20. Ambroxol reverses tau and α-synuclein accumulation in a cholinergic N370S GBA1 mutation model
21. The PINK1—Parkin mitophagy signalling pathway is not functional in peripheral blood mononuclear cells
22. Mitochondrial respiratory chain and Krebs cycle enzyme function in human donor livers subjected to end-ischaemic hypothermic machine perfusion
23. Signal transducer and activator of transcription 2 deficiency is a novel disorder of mitochondrial fission
24. Selective striatal mtDNA depletion in end-stage Huntingtonʼs disease R6/2 mice
25. Increased sensitivity of myoblasts to oxidative stress in amyotrophic lateral sclerosis peripheral tissues
26. Influence of mitochondrial DNA level on cellular energy metabolism: implications for mitochondrial diseases
27. COX10 Mutations Resulting in Complex Multisystem Mitochondrial Disease That Remains Stable Into Adulthood
28. Mitochondria as oncotarget: a comparison between the tetracycline analogs doxycycline and COL-3
29. Relapsing neuropathy in an 18-year-old woman
30. Mutations in the mitochondrial complex I assembly factor NDUFAF1 cause fatal infantile hypertrophic cardiomyopathy
31. Kearns–Sayre syndrome caused by defective R1/p53R2 assembly
32. Chapter 5 - Mitochondrial DNA: Structure, Genetics, Replication and Defects
33. The PINK1 – Parkin mitophagy signalling pathway is not functional in peripheral blood mononuclear cells
34. FOXRED1, encoding an FAD-dependent oxidoreductase complex-I-specific molecular chaperone, is mutated in infantile-onset mitochondrial encephalopathy
35. Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy
36. Clinical, biochemical and morphological features of hepatocerebral syndrome with mitochondrial DNA depletion due to deoxyguanosine kinase deficiency
37. The Human Mitochondrial Genome
38. Status epilepticus in children with Alpersʼ disease caused by POLG1 mutations: EEG and MRI features
39. Analysis of Mutant DNA Polymerase γ in Patients With Mitochondrial DNA Depletion
40. Human Cytochrome c Oxidase: Structure, Function, and Deficiency
41. A LON-ClpP Proteolytic Axis Degrades Complex I to Extinguish ROS Production in Depolarized Mitochondria
42. Mitochondrial DNA expression
43. Myoclonus–dystonia syndrome with severe depression is caused by an exon-skipping mutation in the ε-sarcoglycan gene
44. Chapter 1 Structure and Function of the Mitochondrial Oxidative Phosphorylation System
45. Dominant inheritance of premature ovarian failure associated with mutant mitochondrial DNA polymerase gamma
46. Mutant torsinA, which causes early-onset primary torsion dystonia, is redistributed to membranous structures enriched in vesicular monoamine transporter in cultured human SH-SY5Y cells
47. Replication of mitochondrial DNA occurs throughout the mitochondria of cultured human cells
48. Mitochondrial DNA depletion can be prevented by dGMP and dAMP supplementation in a resting culture of deoxyguanosine kinase-deficient fibroblasts
49. NDUFA4 (Renamed COXFA4) Is a Cytochrome-c Oxidase Subunit
50. Regulation of cytochrome c oxidase by interaction of ATP at two binding sites, one on subunit VIa
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