[HTML][HTML] Pathogenesis and molecular mechanisms of Anderson–Fabry disease and possible new molecular addressed therapeutic strategies

A Tuttolomondo, I Simonetta, R Riolo, F Todaro… - International Journal of …, 2021 - mdpi.com
Anderson–Fabry disease (AFD) is a rare disease with an incidenceof approximately 1:
117,000 male births. Lysosomal accumulation of globotriaosylceramide (Gb3) is the element …

[HTML][HTML] Mitochondrial oxidative stress and cell death in podocytopathies

YT Zhu, C Wan, JH Lin, HP Hammes, C Zhang - Biomolecules, 2022 - mdpi.com
Podocytopathies are kidney diseases that are driven by podocyte injury with proteinuria and
proteinuria-related symptoms as the main clinical presentations. Albeit podocytopathies are …

[HTML][HTML] Implications of sphingolipid metabolites in kidney diseases

SK Mallela, S Merscher, A Fornoni - International journal of molecular …, 2022 - mdpi.com
Sphingolipids, which act as a bioactive signaling molecules, are involved in several cellular
processes such as cell survival, proliferation, migration and apoptosis. An imbalance in the …

Necroptosis inhibitors: mechanisms of action and therapeutic potential

Y Zhou, Z Cai, Y Zhai, J Yu, Q He, Y He, S Jitkaew… - Apoptosis, 2024 - Springer
Necroptosis is a type of programmed cell death that is morphologically similar to necrosis.
This type of cell death is involved in various pathophysiological disorders, including …

Elucidating the toxic effect and disease mechanisms associated with Lyso-Gb3 in Fabry disease

V Nikolaenko, DG Warnock, K Mills… - Human Molecular …, 2023 - academic.oup.com
Fabry disease stems from a deficiency of alpha-galactosidase and results in the
accumulation of globotriaosylceramide (Gb3). However, the production of its deacylated …

[HTML][HTML] Curcumin blocks high glucose-induced podocyte injury via RIPK3-dependent pathway

H Chung, SW Lee, M Hyun, SY Kim, HG Cho… - Frontiers in Cell and …, 2022 - frontiersin.org
Podocyte loss is well known to play a critical role in the early progression of diabetic
nephropathy. A growing number of studies are paying attention to necroptosis, a …

[HTML][HTML] Exploring Pro-Inflammatory Immunological Mediators: Unraveling the Mechanisms of Neuroinflammation in Lysosomal Storage Diseases

MK Pandey - Biomedicines, 2023 - mdpi.com
Lysosomal storage diseases are a group of rare and ultra-rare genetic disorders caused by
defects in specific genes that result in the accumulation of toxic substances in the lysosome …

[HTML][HTML] Normal and Dysregulated Sphingolipid Metabolism: Contributions to Podocyte Injury and Beyond

M Tolerico, S Merscher, A Fornoni - Cells, 2024 - mdpi.com
Podocyte health is vital for maintaining proper glomerular filtration in the kidney.
Interdigitating foot processes from podocytes form slit diaphragms which regulate the …

[HTML][HTML] EW-7197 Attenuates the Progression of Diabetic Nephropathy in db/db Mice through Suppression of Fibrogenesis and Inflammation

KB Ha, W Sangartit, AR Jeong, ES Lee… - Endocrinology and …, 2022 - synapse.koreamed.org
Background Diabetic nephropathy (DN) is characterized by albuminuria and accumulation
of extracellular matrix (ECM) in kidney. Transforming growth factor-β (TGF-β) plays a central …

[HTML][HTML] The role of tubular cells in the pathogenesis of Fabry nephropathy

P Rozenfeld, S Feriozzi, F Braun - Frontiers in Cardiovascular …, 2024 - frontiersin.org
The pathophysiology of Fabry nephropathy (FN) is induced by galactosidase A deficiency
with a chronic exposure of glycolipids to every lineage of renal cells. Tissue damage is …