Cargando…

Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention

Mitochondria are small cellular constituents that generate cellular energy (ATP) by oxidative phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous group of multisystemic disorders, including diabetes, cancer, ageing-related pathologies and rare mitochondrial disease...

Descripción completa

Detalles Bibliográficos
Autores principales:	van de Wal, Melissa A E, Adjobo-Hermans, Merel J W, Keijer, Jaap, Schirris, Tom J J, Homberg, Judith R, Wieckowski, Mariusz R, Grefte, Sander, van Schothorst, Evert M, van Karnebeek, Clara, Quintana, Albert, Koopman, Werner J H
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Oxford University Press 2021
Materias:	Review Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8967107/ https://www.ncbi.nlm.nih.gov/pubmed/34849584 http://dx.doi.org/10.1093/brain/awab426

Ejemplares similares

Microglial response promotes neurodegeneration in the Ndufs4 KO mouse model of Leigh syndrome
por: Aguilar, Kevin, et al.
Publicado: (2022)

Double administration of self-complementary AAV9(NDUFS4) prevents Leigh disease in Ndufs4(−/−) mice
por: Corrà, Samantha, et al.
Publicado: (2022)

Peripheral macrophages drive CNS disease in the Ndufs4(−/−) model of Leigh syndrome
por: Hanaford, Allison R., et al.
Publicado: (2023)

Leukocytes mediate disease pathogenesis in the Ndufs4(KO) mouse model of Leigh syndrome
por: Stokes, Julia C., et al.
Publicado: (2022)

Measuring Locomotor Activity and Behavioral Aspects of Rodents Living in the Home-Cage
por: Klein, Christian J. M. I., et al.
Publicado: (2022)

Corrigendum: Measuring Locomotor Activity and Behavioral Aspects of Rodents Living in the Home-Cage
por: Klein, Christian J. M. I., et al.
Publicado: (2022)

Loss of Mitochondrial Ndufs4 in Striatal Medium Spiny Neurons Mediates Progressive Motor Impairment in a Mouse Model of Leigh Syndrome
por: Chen, Byron, et al.
Publicado: (2017)

Normobaric hypoxia shows enhanced FOXO1 signaling in obese mouse gastrocnemius muscle linked to metabolism and muscle structure and neuromuscular innervation
por: Song, Jingyi, et al.
Publicado: (2023)

The p.M292T NDUFS2 mutation causes complex I-deficient Leigh syndrome in multiple families
por: Tuppen, Helen A. L., et al.
Publicado: (2010)

Nutrigenomics of Body Weight Regulation: A Rationale for Careful Dissection of Individual Contributors
por: Keijer, Jaap, et al.
Publicado: (2014)

Effects of cold exposure revealed by global transcriptomic analysis in ferret peripheral blood mononuclear cells
por: Reynés, Bàrbara, et al.
Publicado: (2019)

Cold Induced Depot-Specific Browning in Ferret Aortic Perivascular Adipose Tissue
por: Reynés, Bàrbara, et al.
Publicado: (2019)

Body Weight Cycling with Identical Diet Composition Does Not Affect Energy Balance and Has No Adverse Effect on Metabolic Health Parameters
por: Palm, Inge F., et al.
Publicado: (2017)

Metabolic effects of the dietary monosaccharides fructose, fructose–glucose, or glucose in mice fed a starch‐containing moderate high–fat diet
por: Bouwman, Lianne M. S., et al.
Publicado: (2020)

Metabolic Response of Visceral White Adipose Tissue of Obese Mice Exposed for 5 Days to Human Room Temperature Compared to Mouse Thermoneutrality
por: van der Stelt, Inge, et al.
Publicado: (2017)

A Constraint-Based Model Analysis of Enterocyte Mitochondrial Adaptation to Dietary Interventions of Lipid Type and Lipid Load
por: Sinha, Neeraj, et al.
Publicado: (2018)

Direct and Long-Term Metabolic Consequences of Lowly vs. Highly-Digestible Starch in the Early Post-Weaning Diet of Mice
por: Fernández-Calleja, José M. S., et al.
Publicado: (2018)

Extended indirect calorimetry with isotopic CO(2) sensors for prolonged and continuous quantification of exogenous vs. total substrate oxidation in mice
por: Fernández-Calleja, José M. S., et al.
Publicado: (2019)

Signaling efficiency of Gαq through its effectors p63RhoGEF and GEFT depends on their subcellular location
por: Goedhart, Joachim, et al.
Publicado: (2013)

Network-based integration of molecular and physiological data elucidates regulatory mechanisms underlying adaptation to high-fat diet
por: Derous, Davina, et al.
Publicado: (2015)

Replacing Part of Glucose with Galactose in the Postweaning Diet Protects Female But Not Male Mice from High-Fat Diet–Induced Adiposity in Later Life
por: Bouwman, Lianne M S, et al.
Publicado: (2019)

Predicting the murine enterocyte metabolic response to diets that differ in lipid and carbohydrate composition
por: Sinha, Neeraj, et al.
Publicado: (2017)

Exploring the associations between transcript levels and fluxes in constraint-based models of metabolism
por: Sinha, Neeraj, et al.
Publicado: (2021)

No Adverse Programming by Post‐Weaning Dietary Fructose of Body Weight, Adiposity, Glucose Tolerance, or Metabolic Flexibility
por: Bouwman, Lianne M. S., et al.
Publicado: (2017)

A Lowly Digestible-Starch Diet after Weaning Enhances Exogenous Glucose Oxidation Rate in Female, but Not in Male, Mice
por: Fernández-Calleja, José M. S., et al.
Publicado: (2019)

Mito-Nuclear Communication by Mitochondrial Metabolites and Its Regulation by B-Vitamins
por: Janssen, Joëlle J. E., et al.
Publicado: (2019)

Mitochondrial complex I deficiency leads to the retardation of early embryonic development in Ndufs4 knockout mice
por: Wang, Mei, et al.
Publicado: (2017)

Galactose in the Post-Weaning Diet Programs Improved Circulating Adiponectin Concentrations and Skeletal Muscle Insulin Signaling
por: Sun, Peixin, et al.
Publicado: (2022)

Lip cyanosis as the first symptom of Leigh syndrome associated with mitochondrial complex I deficiency due to a compound heterozygous NDUFS1 mutation: A case report
por: Men, Lina, et al.
Publicado: (2022)

Interference of flavonoids with enzymatic assays for the determination of free fatty acid and triglyceride levels
por: Hoek-van den Hil, Elise F., et al.
Publicado: (2011)

A Difference in Fatty Acid Composition of Isocaloric High-Fat Diets Alters Metabolic Flexibility in Male C57BL/6JOlaHsd Mice
por: Duivenvoorde, Loes P. M., et al.
Publicado: (2015)

Sensitive Detection of p65 Homodimers Using Red-Shifted and Fluorescent Protein-Based FRET Couples
por: Goedhart, Joachim, et al.
Publicado: (2007)

Transcriptional Response of White Adipose Tissue to Withdrawal of Vitamin B3
por: Shi, Wenbiao, et al.
Publicado: (2019)

Quantitative Co-Expression of Proteins at the Single Cell Level – Application to a Multimeric FRET Sensor
por: Goedhart, Joachim, et al.
Publicado: (2011)

Endurance Exercise Increases Intestinal Uptake of the Peanut Allergen Ara h 6 after Peanut Consumption in Humans
por: JanssenDuijghuijsen, Lonneke M., et al.
Publicado: (2017)

The opposite prognostic effect of NDUFS1 and NDUFS8 in lung cancer reflects the oncojanus role of mitochondrial complex I
por: Su, Chia-Yi, et al.
Publicado: (2016)

A Single Intravenous Injection of AAV-PHP.B-hNDUFS4 Ameliorates the Phenotype of Ndufs4(−/−) Mice
por: Silva-Pinheiro, Pedro, et al.
Publicado: (2020)

A microarray-based approach to evaluate the functional significance of protein-binding motifs
por: Sinzinger, Michael D., et al.
Publicado: (2016)

Induction of lipid oxidation by polyunsaturated fatty acids of marine origin in small intestine of mice fed a high-fat diet
por: van Schothorst, Evert M, et al.
Publicado: (2009)

Neuroacanthocytosis: Observations, Theories and Perspectives on the Origin and Significance of Acanthocytes
por: Adjobo-Hermans, Merel J. W., et al.
Publicado: (2015)

Cannot write session to /tmp/vufind_sessions/sess_acrrou7top3mkuo4ou401l6u1p