Results 81 to 90 of about 4,693,382 (193)

Alpha methyl acyl CoA racemase deficiency: Diagnosis with isolated elevated liver enzymes

The Turkish Journal of Pediatrics, 2019
Alpha methy acyl CoA racemase (AMACR) deficiency is a rare autosomal recessive peroxisomal disorder characterized by cholestatic liver disease in the neonatal period, and variable neurologic symptoms affecting central and peripheral nervous systems in ...
Mehmet Gündüz, Özlem Ünal, Aynur Küçükçongar-Yavaş, Çiğdem Kasapkara   +3 more
doaj   +1 more source

Functional characterisation of peroxisomal β-oxidation disorders in fibroblasts using lipidomics

Journal of Inherited Metabolic Disease, 2017
Peroxisomes play an important role in a variety of metabolic pathways, including the α- and β-oxidation of fatty acids, and the biosynthesis of ether phospholipids.
Katharina Herzog, Mia L. Pras-Raves, S. Ferdinandusse, M. Vervaart, Angela C. M. Luyf, A. V. van Kampen, R. Wanders, H. Waterham, F. Vaz   +8 more
semanticscholar   +1 more source

The Role of Oxidative Stress in Periodontitis

Journal of Periodontal Research, EarlyView.
Oxidative stress is involved in multiple chemical reactions that take place in different intracellular organelles: mitochondria, rough endoplasmic reticulum, peroxisomes, autophagy, and aging, and can be influenced by exogenous factors: nutrition, physical activity, psychological status, environmental conditions, microbiome, and drugs.
Pedro Bullon, Francesca Giampieri, Beatriz Bullon, Maurizio Battino   +3 more
wiley   +1 more source

Evolutionary Conserved and Divergent Responses to Copper Zinc Superoxide Dismutase Inhibition in Plants

Plant, Cell &Environment, EarlyView.
ABSTRACT After an initial evolution in a reducing environment, life got successively challenged by reactive oxygen species (ROS), especially during the great oxidation event (GOE) that followed the development of photosynthesis. Therefore, ROS are deeply intertwined into the physiological, morphological and transcriptional responses of most present‐day
Stephanie Frohn, Fabian B. Haas, Benjamin G. Chavez, Bernd H. Dreyer, Erik V. Reiss, Anne Ziplys, Heiko Weichert, Saskia Hiltemann, José M. Ugalde, Andreas J. Meyer, John C. D'Auria, Stefan A. Rensing, Jos H. M. Schippers   +12 more
wiley   +1 more source

Tripartite Interaction Between Penicillium pinophilum‐Host Plants and CMV‐Y: A Model for Endophyte‐Mediated Viral Biocontrol

Plant, Cell &Environment, EarlyView.
ABSTRACT Plant‐fungus‐virus tripartite interactions represent complex ecological systems in which mutualistic endophytes can influence host physiology, yet the molecular basis of endophyte‐mediated defence remains poorly understood. Here, we demonstrate that the endophytic fungus Penicillium pinophilum EU0013 suppresses the yellow strain of cucumber ...
Sarah R. Ibiang, Ivan Galis, Hideki Kondo, Nobuhiro Suzuki   +3 more
wiley   +1 more source

Prenatal and perinatal diagnosis of peroxisomal disorders

, 1989
Peroxisomes play an essential role in human cellular metabolism. Peroxisomal disorders, a group of genetic diseases caused by peroxisomal dysfunction, can be classified into three groups: (1) disorders of peroxisome biogenesis with a generalized loss of ...
Schutgens, R. B., R. J. A. Wanders, H. van den Bosch, Schrakamp, G., Heymans, H. S., J. M. Tager, H. S. A. Heymans, Tager, J. M., R. B. H. Schutgens, Wanders, R. J., van den Bosch, H., G. Schrakamp   +11 more
core   +1 more source

Clinical and Biochemical Features in a Patient With Mitochondrial Fission Factor Gene Alteration

Frontiers in Genetics, 2018
Mitochondrial Fission Factor (MFF) is part of a protein complex that promotes mitochondria and peroxisome fission. Hitherto, only 5 patients have been reported harboring mutations in MFF, all of them with the clinical features of a very early onset Leigh-
Alessia Nasca, Francesca Nardecchia, Anna Commone, Michela Semeraro, Andrea Legati, Barbara Garavaglia, Daniele Ghezzi, Daniele Ghezzi, Vincenzo Leuzzi   +8 more
doaj   +1 more source

Periodontitis and metabolic dysfunction‐associated steatotic liver disease

Periodontology 2000, EarlyView.
Abstract Objective Periodontitis is a chronic inflammatory disease with systemic effects that extend beyond the oral cavity and contribute to systemic immune and metabolic dysregulation. Chronic liver diseases, particularly metabolic dysfunction‐associated steatotic liver disease (MASLD) and its progressive phenotypes, have emerged as major global ...
Rafael Scaf de Molon, Erica Dorigatti de Avila, Davi da Silva Barbirato, Mariana Fampa Fogacci, Jamil Awad Shibli, Anton Sculean, Alpdogan Kantarci   +6 more
wiley   +1 more source

Canonical and non‐canonical functions of proteins regulating mitochondrial dynamics in mammalian physiology

The Journal of Physiology, EarlyView.
Abstract figure legend Mitochondria are highly dynamic organelles that continuously remodel their architecture through coordinated cycles of fusion and fission. This review examines the four key GTPases that orchestrate mitochondrial dynamics in mammals: MFN1, MFN2, OPA1, and DRP1.
Rémi Chaney, Vincent Marcangeli, Krystel Desjardins, Shima Taherkhani, Gilles Gouspillou   +4 more
wiley   +1 more source

Metabolomics to study functional consequences in peroxisomal disorders [PDF]

, 2017
This thesis focusses on metabolomics approaches performed in cultured cells and blood samples from patients with peroxisomal disorders. By applying both targeted and untargeted metabolomics, the aim of these approaches was to study the functional ...
Herzog, Katharina, Herzog, K.
core   +21 more sources