Results 91 to 100 of about 2,687,044 (278)
Inhibition of PIKfyve Leads to Lysosomal Disorders via Dysregulation of mTOR Signaling
CellsPIKfyve is an endosomal lipid kinase that synthesizes phosphatidylinositol 3,5-biphosphate from phosphatidylinositol 3-phsphate. Inhibition of PIKfyve activity leads to lysosomal enlargement and cytoplasmic vacuolation, attributed to impaired lysosomal ...
Jianhong Xia +3 more
doaj +1 more source
High lumenal chloride in the lysosome is critical for lysosome function
eLife, 2017 Lysosomes are organelles responsible for the breakdown and recycling of cellular machinery. Dysfunctional lysosomes give rise to lysosomal storage disorders as well as common neurodegenerative diseases.
Kasturi Chakraborty +2 more
doaj +1 more source
Advanced Science, EarlyView.This review elucidates how cancer cell metabolic reprogramming—across glucose, lipid, amino acid, and nucleotide pathways—remodels the tumor microenvironment to suppress anti‐tumor immunity and promote immune escape. Targeting these metabolic axes offers promising strategies to overcome immunotherapy resistance and enhance cancer treatment.
Guoqing Xiang +5 more
wiley +1 more source
Risks of long-term port use in enzyme replacement therapy for lysosomal storage disorders
Molecular Genetics and Metabolism Reports, 2018 Totally implantable vascular access devices (TIVADs) are commonly used in conjunction with enzyme replacement therapy (ERT) for lysosomal storage disorders (LSDs).
Christian J. Hendriksz +4 more
doaj +1 more source
Lysosomal storage diseases as disorders of autophagy
, 2008 The cellular turnover of proteins and organelles requires cooperation between the autophagic and the lysosomal degradation pathways. A crucial step in this process is the fusion of the autophagosome with the lysosome.
Rubinsztein D.C. +3 more
core
FDA orphan drug designations for lysosomal storage disorders – a cross-sectional analysis - Fig 2
, 2020 A: Orphan drug designations granted by the FDA for compounds intended to treat lysosomal storage disorders by year and specific disease. B: Orphan drug designations granted by the FDA for compounds intended to treat lysosomal storage disorders by year ...
Sven F. Garbade (3700849) +7 more
core +1 more source
Advanced Science, EarlyView.This graphical abstract illustrates how chaperone‐mediated autophagy (CMA) regulates intervertebral disc degeneration (IDD). Under normal homeostasis (B), CMA degrades cytoplasmic Midnolin (MIDN) to maintain proteostasis. Under inflammatory stress (A), impaired CMA leads to cytoplasmic MIDN accumulation.
Xianglong Chen +9 more
wiley +1 more source
Newborn Screening for Lysosomal Storage Disorders in Belgium
Journal of Inborn Errors of Metabolism and Screening, 2017 Lysosomal storage disorders (LSDs) are a group of metabolic disorders with various clinical presentations, which complicate diagnosis. A pilot study was performed to test the appropriateness and effectiveness of the newborn screening method for Pompe ...
Francois Eyskens MD, PhD +1 more
doaj +1 more source
Immune system irregularities in lysosomal storage disorders
, 2008 Lysosomal storage disorders (LSDs) are genetically inherited diseases characterized by the accumulation of disease-specific biological materials such as proteolipids or metabolic intermediates within the lysosome.
Ming J. Lim +7 more
core +1 more source
OAF Blocks SIAH1‐Mediated Degradation of SCPX, a Therapeutic Strategy for MASLD
Advanced Science, EarlyView.This study shows OAF directly binds to SCPX and inhibits its interaction with the E3 ligase SIAH1, thereby protecting SCPX from ubiquitin‐dependent degradation and stabilizing its levels. This OAF‐SCPX axis represents a novel pathway in lipid homeostasis, highlighting OAF as a promising therapeutic candidate for MASLD.
Zongxi Li +11 more
wiley +1 more source