Results 51 to 60 of about 1,135,071 (192)

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

Multiple Teeth Impaction in an Adult Patient Affected by Infantile Refsum Disease: A Case Report

Oral
Introduction: Infantile Refsum disease (IRD) is considered one of the milder phenotypes within the Zellweger Spectrum Disorders (ZSDs), a group of peroxisomal biogenesis disorders characterized by a generalized impairment of peroxisomal function ...
Edoardo Staderini, Gioele Gioco, Federica Guglielmi, Francesca Cazzato, Licia Leccese, Carmen Chiara Nacca, Patrizia Gallenzi   +6 more
doaj   +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

Reinvestigation of trihydroxycholestanoic acidemia reveals a peroxisome biogenesis disorder

, 2004
OBJECTIVE: To determine the enzymatic defect in a patient with ataxia, dysarthric speech, dry skin, hypotonia, and absent reflexes. The patient was previously diagnosed with a presumed deficiency of trihydroxycholestanoyl-CoA oxidase.
Gootjes, J., Ferdinandusse, S., Skovby, F., Christensen, E., Wanders, R. J. A.   +4 more
core   +1 more source

Mutations in SRD5B1 (AKR1D1), the gene encoding Delta(4)-3-oxosteroid 5 beta-reductase, in hepatitis and liver failure in infancy [PDF]

, 2003
Background: A substantial group of patients with cholestatic liver disease in infancy excrete, as the major urinary bile acids, the glycine and taurine conjugates of 7alpha-hydroxy-3-oxo-4-cholenoic acid and 7alpha, 12alpha-dihydroxy-3- oxo-4-cholenoic ...
Duran, M., Scambler, PJ, Custard, E. J., Bouquet, J, Lemonde, H. A., Clayton, P. T., Duran, M, Overmars, H, Bouquet, J., Scambler, P.J., Lemonde, H., Clayton, P.T., Custard, EJ, Scambler, P. J., Clayton, PT, Lemonde, HA, Custard, E.J., Overmars, H.   +17 more
core   +2 more sources

Relaxin‐2: Shaping the Proteomic Landscape of Skeletal Muscle Physiology, Glucose Trafficking, and Mitochondrial Function in Rat

The FASEB Journal, Volume 40, Issue 18, 30 September 2026.
Cardioprotective hormone relaxin‐2 showed relevant effects on rat skeletal muscle by altering proteins linked to muscle function, regeneration, differentiation, mitochondrial function, glucose metabolism, and structural integrity and organization. Specifically, relaxin‐2 reduced the expression of 95 proteins, increased 32, and elicited unique proteins ...
Xocas Vázquez‐Abuín, Lucía Gómez‐Cibeira, Javier García‐Seara, Pablo de la Fuente‐López, Carlos Minguito‐Carazo, Susana B. Bravo, Manuel Otero‐Santiago, Oreste Gualillo, José Ramón González‐Juanatey, Francisca Lago, Sandra Feijóo‐Bandín, Alana Aragón‐Herrera   +11 more
wiley   +1 more source

Association between peroxisomal biogenesis factor 7 and autism spectrum disorders in a Korean population

, 2018
Autism spectrum disorder is a neurodevelopmental disorder characterized by deficits in social communication, impaired reciprocal social interaction, and repetitive patterns of behaviors or interests. Although the cause of autism spectrum disorder remains
방희정
core   +1 more source

From Single Cells to Diagnosis: Proteomics Technologies in the Multi‐Omics Landscape of Rare and Mitochondrial Diseases

Journal of Inherited Metabolic Disease, Volume 49, Issue 5, September 2026.
Wetzel et al. outline how individual omics methods contribute to the diagnosis of patients with rare, and particularly mitochondrial diseases, with a focus on how spatial proteomics is joining this multi‐omics stack. ABSTRACT Proteomics by mass spectrometry has rapidly matured from a niche method into a standard tool.
Simon Wetzel, Sebastian Porsdam Mann, Florian A. Rosenberger   +2 more
wiley   +1 more source

The Dynamic Alliance of p53 and Metabolism in the Tumor Microenvironment Shapes Tumor Evolution

BioEssays, Volume 48, Issue 8, August 2026.
p53 is an adaptive processor positioned at the interface between metabolism and the tumor microenvironment. By integrating nutrient, mechanical, inflammatory, and redox cues while reciprocally remodeling metabolism, stromal and immune compartments, and cancer cell plasticity, WT and mutant p53 orchestrate cell competition, tumor evolution, metastasis ...
Sebastien M. Joruiz, Francesco Napoletano, Rebecca Bertolio, Giannino Del Sal   +3 more
wiley   +1 more source

SIRT Family: Biological Functions and Therapeutic Targets

MedComm, Volume 7, Issue 8, August 2026.
SIRT1–SIRT7 networks from transgenic mice to human‑relevant therapeutic targets. SIRT1–SIRT7 form an isoform‑, organ‑, and disease‑specific regulatory network. Transgenic Sirt1–7 mouse models define central regulatory SIRTs (SIRT1, SIRT3, SIRT6), context‑dependent modifiers (SIRT2, SIRT4, SIRT5, SIRT7), and their key mechanisms and target organs. These
Jia‐Yi Wang, Feng‐Li Jiang, Fang‐Yuan Zhang, Dong‐Hui Huang, Xiao‐Ying Li, Song Gao, Hua You, Qi‐Jun Wu, Huan‐Huan Chen, Ting‐Ting Gong   +9 more
wiley   +1 more source