Neurophysiological, behavioral and morphological abnormalities in the Fabry knockout mice
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 +7 more
doaj +1 more source
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 +9 more
doaj +1 more source
A Peroxisomal Lon Protease and Peroxisome Degradation by Autophagy Play Key Roles in Vitality of Hansenula polymorpha Cells [PDF]
In eukaryote cells various mechanisms exist that are responsible for the removal of non-functional proteins. Here we show that in the yeast Hansenula polymorpha (H.
Jourdan, Stefanie +6 more
core +2 more sources
No peroxisome is an island — Peroxisome contact sites
In order to optimize their multiple cellular functions, peroxisomes must collaborate and communicate with the surrounding organelles. A common way of communication between organelles is through physical membrane contact sites where membranes of two organelles are tethered, facilitating exchange of small molecules and intracellular signaling.
Shai, Nadav +2 more
openaire +2 more sources
A comparative study of peroxisomal structures in Hansenula polymorpha pex mutants [PDF]
In a recent study, we performed a systematic genome analysis for the conservation of genes involved in peroxisome biogenesis (PEX genes) in various fungi.
Koek, Anne, +8 more
core +2 more sources
ISOC1 Modulates Inflammatory Responses in Macrophages through the AKT1/PEX11B/Peroxisome Pathway
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 +6 more
doaj +1 more source
Cytoprotective and Antioxidants in Peroxisomal Neurodegenerative Diseases
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 +9 more
doaj +1 more source
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 +10 more
doaj +1 more source
Regulation of skeletal muscle cell plasticity by the peroxisome proliferator-activated receptor gamma coactivator 1alpha [PDF]
Exercise triggers a pleiotropic response in skeletal muscle, which results in a profound remodeling of this tissue. Physical activity-dependent muscle fiber plasticity is regulated by a number of distinct signaling pathways.
Handschin, C.
core +1 more source
Characterization of the Peroxisomal Proteome and Redox Balance in Human Prostate Cancer Cell Lines
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 +5 more
doaj +1 more source

