Results 21 to 30 of about 162,956 (297)

Reduced peroxisomal import triggers peroxisomal retrograde signaling [PDF]

Cell Reports, 2021
Maintaining organelle function in the face of stress is known to involve organelle-specific retrograde signaling. Using Caenorhabditis elegans, we present evidence of the existence of such retrograde signaling for peroxisomes, which we define as the peroxisomal retrograde signaling (PRS).
Rackles, Elisabeth, Witting, Michael, Forné, Ignasi, Zhang, Xing, Zacherl, Judith, Schrott, Simon, Fischer, Christian, Ewbank, Jonathan, Osman, Christof, Imhof, Axel, Rolland, Stéphane   +10 more
openaire   +5 more sources

Neurophysiological, behavioral and morphological abnormalities in the Fabry knockout mice

Neurobiology of Disease, 2009
Fabry disease (OMIM 301500) is a rare X-linked recessive disorder caused by mutations in the α-galactosidase gene (GLA). Loss of α-galactosidase (α-Gal) activity leads to the abnormal accumulation of glycosphingolipids in lysosomes predominantly of ...
L.G. Rodrigues, M.J. Ferraz, D. Rodrigues, M. Pais-Vieira, D. Lima, R.O. Brady, M.M. Sousa, M.C. Sá-Miranda   +7 more
doaj   +1 more source

Exercise, Nutrition, and Supplements in the Muscle Carnitine Palmitoyl-Transferase II Deficiency: New Theoretical Bases for Potential Applications

Frontiers in Physiology, 2021
Carnitine palmitoyltransferase II (CPTII) deficiency is the most frequent inherited disorder regarding muscle fatty acid metabolism, resulting in a reduced mitochondrial long-chain fatty acid oxidation during endurance exercise. This condition leads to a
Massimo Negro, Giuseppe Cerullo, Mauro Parimbelli, Alberto Ravazzani, Fausto Feletti, Angela Berardinelli, Hellas Cena, Hellas Cena, Giuseppe D’Antona, Giuseppe D’Antona   +9 more
doaj   +1 more source

ISOC1 Modulates Inflammatory Responses in Macrophages through the AKT1/PEX11B/Peroxisome Pathway

Molecules, 2022
Inflammation underlies a variety of physiological and pathological processes and plays an essential role in shaping the ensuing adaptive immune responses and in the control of pathogens.
Xiaoyuan Lin, Qingting Zhao, Beibei Fu, Yan Xiong, Shanfu Zhang, Shiyao Xu, Haibo Wu   +6 more
doaj   +1 more source

Peroxisomal Diseases

Annals of Medicine, 1992
The peroxisomal diseases, which are rare inborn metabolic errors, often have serious effects on the well being of the individual and many of them are fatal at an early age. The Zellweger cerebro-hepato-renal syndrome represents a group consisting of diseases with a generalized loss of peroxisomal functions and is considered as a prototype for ...
P M, Palosaari, J M, Kilponen, J K, Hiltunen   +2 more
openaire   +2 more sources

Cytoprotective and Antioxidants in Peroxisomal Neurodegenerative Diseases

Proceedings, 2019
Several of the peroxisomal neurodegenerative disorders are the consequence of a specific deficiency of an enzyme or a transporter involved in peroxisomal beta-oxidation of very long chain fatty acids [1,2].
Mustapha Cherkaoui-Malki, Saad Shaaban, Mounia Tahri-Joutey, Ahmed Elshobaky, Fatima-Ezzahra Saih, Dominique Vervandier-Fasseur, Claus Jacob, Boubker Nasser, Norbert Latruffe, Pierre Andreoletti   +9 more
doaj   +1 more source

Peroxisomes Regulate Cellular Free Fatty Acids to Modulate Mast Cell TLR2, TLR4, and IgE-Mediated Activation

Frontiers in Cell and Developmental Biology, 2022
Mast cells are specialized, tissue resident, immune effector cells able to respond to a wide range of stimuli. MCs are involved in the regulation of a variety of physiological functions, including vasodilation, angiogenesis and pathogen elimination.
Dihia Meghnem, Dihia Meghnem, Dihia Meghnem, Edwin Leong, Marinella Pinelli, Marinella Pinelli, Jean S. Marshall, Jean S. Marshall, Jean S. Marshall, Francesca Di Cara, Francesca Di Cara   +10 more
doaj   +1 more source

Characterization of the Peroxisomal Proteome and Redox Balance in Human Prostate Cancer Cell Lines

Antioxidants
Prostate cancer (PCa) is associated with disruptions in cellular redox balance. Given the intricate role of peroxisomes in redox metabolism, we conducted comprehensive proteomics analyses to compare peroxisomal and redox protein profiles between benign ...
Mohamed A. F. Hussein, Celien Lismont, Cláudio F. Costa, Hongli Li, Frank Claessens, Marc Fransen   +5 more
doaj   +1 more source

Enterovirus 71 induces neural cell apoptosis and autophagy through promoting ACOX1 downregulation and ROS generation

Virulence, 2020
Enterovirus 71 (EV71) infection causes hand, foot, and mouth disease (HFMD), and even fatal neurological complications. However, the mechanisms underlying EV71 neurological pathogeneses are largely unknown.
Lei You, Junbo Chen, Weiyong Liu, Qi Xiang, Zhen Luo, Wenbiao Wang, Wei Xu, Kailang Wu, Qi Zhang, Yingle Liu, Jianguo Wu   +10 more
doaj   +1 more source

Membrane Processing and Steady-State Regulation of the Alternative Peroxisomal Import Receptor Pex9p

Frontiers in Cell and Developmental Biology, 2020
Import of peroxisomal matrix proteins with a type 1 peroxisomal targeting signal (PTS1) in Saccharomyces cerevisiae is facilitated by cytosolic import receptors Pex5p and Pex9p. While Pex5p has a broad specificity for all PTS1 proteins independent of the
Markus Rudowitz, Ralf Erdmann, Wolfgang Schliebs   +2 more
doaj   +1 more source