Results 31 to 40 of about 36,163 (246)

Modeling Lysosomal Storage Diseases in the Zebrafish

Frontiers in Molecular Biosciences, 2020
Lysosomal storage diseases (LSDs) are a family of 70 metabolic disorders characterized by mutations in lysosomal proteins that lead to storage material accumulation, multiple-organ pathologies that often involve neurodegeneration, and early mortality in ...
T. Zhang, R. T. Peterson
doaj   +1 more source

Synaptic Function and Dysfunction in Lysosomal Storage Diseases

Frontiers in Cellular Neuroscience, 2021
Lysosomal storage diseases (LSDs) with neurological involvement are inherited genetic diseases of the metabolism characterized by lysosomal dysfunction and the accumulation of undegraded substrates altering glial and neuronal function.
Rima Rebiai, Maria I. Givogri, Swetha Gowrishankar, Stephania M. Cologna, Simon T. Alford, Ernesto R. Bongarzone   +5 more
doaj   +1 more source

Relationship of Lysosomal Storage Diseases (LSD) with Autophagy

Van Tıp Dergisi, 2022
Lysosomes are organelles that degrade damaged components or structures that have completed their functions and have roles in the last step of the autophagy pathway. Damage of the autophagy-lysosome pathway can cause vital problems for the cell. Lysosomal
Seda Keskin, İsmail Mert Alkaç, Burcu Çerçi   +2 more
doaj   +1 more source

Lysosomes, Lysosomal Storage Diseases, and Inflammation

Journal of Inborn Errors of Metabolism and Screening, 2016
Lysosomes were originally described in the early 1950s by de Duve who was also the first to recognize the importance of these organelles in human disease.
Calogera M. Simonaro PhD
doaj   +1 more source

Cell surface interactome analysis identifies TSPAN4 as a negative regulator of PD‐L1 in melanoma

Molecular Oncology, EarlyView.
Using cell surface proximity biotinylation, we identified tetraspanin TSPAN4 within the PD‐L1 interactome of melanoma cells. TSPAN4 negatively regulates PD‐L1 expression and lateral mobility by limiting its interaction with CMTM6 and promoting PD‐L1 degradation.
Guus A. Franken, Andrea Abel Gutierrez, Imke van Rossum, Cornelia G. Spruijt, Michiel Vermeulen, Guido van Mierlo, Blanca Scheijen, Annemiek B. van Spriel   +7 more
wiley   +1 more source

Types and Genetic Evaluation of Lysosomal Storage Diseases in Kurdistan Region

مجلة الكوفة الطبية
Background and objectives: Lysosomal storage diseases are a set of single-gene disorders that is attributed to insufficient certain lysosomal hydrolase activity or non-enzymatic proteins vital for typical lysosomal functions.
Lana Ahmed Mohammed
doaj   +1 more source

Deciphering transcriptional plasticity in pancreatic ductal adenocarcinoma reveals alterations in sensory neuron innervation

Molecular Oncology, EarlyView.
Pancreatic sensory neurons innervating healthy and PDAC tissue were retrogradely labeled and profiled by single‐cell RNA sequencing. Tumor‐associated innervation showed a dominant neurofilament‐positive subtype, altered mitochondrial gene signatures, and reduced non‐peptidergic neurons.
Elena Genova, Michele Montrone, Uday Rangaswamy, Francesco Diversi, Irene Schiavo, Denise Ferrarini, Roberta Di Florio, Irene Longo, Michele Coscia, Nicola Zamboni, Giorgia Demontis, Lisa Veghini, Vincenzo Corbo, Remo Sanges, Paul Heppenstall   +14 more
wiley   +1 more source

The role of lipid metabolism in neuronal senescence

FEBS Open Bio, EarlyView.
Disrupted lipid metabolism, through alterations in lipid species or lipid droplet accumulation, can drive neuronal senescence. However, lipid dyshomeostasis can also occur alongside neuronal senescence, further amplifying tissue damage. Delineating how lipid‐induced senescence emerges in neurons and glial cells, and how it contributes to ageing and ...
Dikaia Tsagkari, Eleftheria Panagiotidou, Nektarios Tavernarakis   +2 more
wiley   +1 more source

Dapagliflozin prevents methylglyoxal‐induced retinal cell death in ARPE‐19 cells

FEBS Open Bio, EarlyView.
Diabetic macular oedema is a diabetes complication of the eye, which may lead to permanent blindness. ARPE‐19 are human retinal cells used to study retinal diseases and potential therapeutics. Methylglyoxal is a compound increased in uncontrolled diabetes due to elevated blood glucose.
Naina Trivedi, Zainab Quraishi, Shima Khezri Azizi Far, Madaleine Ward, Aidan Conway, Reem Abdulredha, Eman Mshari, Yousif A. Shamsaldeen   +7 more
wiley   +1 more source

Cutaneous Melanoma Drives Metabolic Changes in the Aged Bone Marrow Immune Microenvironment

Aging and Cancer, EarlyView.
Melanoma, the deadliest form of skin cancer, increasingly affects older adults. Our study reveals that melanoma induces changes in iron and lipid levels in the bone marrow, impacting immune cell populations and increasing susceptibility to ferroptosis.
Alexis E. Carey, Murilo Ramos Rocha, Cheyenne Palm, Vania Wang, Denys Balandin, Ethan Black, Kevin Y. Zhang, Fan Huang, Daniel J. Zabransky, Yash Chhabra, Mareike Peters, Gabriel Ghiaur, Ashani Weeraratna   +12 more
wiley   +1 more source