Results 61 to 70 of about 5,931 (183)
Prosaposin Is Cleaved Into Saposins by Multiple Cathepsins in a Progranulin‐Regulated Fashion
Prosaposin (PSAP) is a lysosomal protein cleaved into four bioactive saposins (SapA‐D) that regulate sphingolipid breakdown. Here, we identify nine cathepsins, including seven newly implicated enzymes, that process PSAP in a pH‐dependent manner to generate distinct cleavage products.
Molly Hodul +7 more
wiley +1 more source
Neurological diseases and stem cell transplantation - review paper [PDF]
Stem cells and their potentials for therapy are major areas of research. The literature on the subject is expanding at a very rapid pace and the great prospectives offered by these remarkable cells are continuously being unravelled.
Blundell, Renald, Vassallo, J.
core
The Influence of Schwann Cell Metabolism and Dysfunction on Axon Maintenance
Main Points Sensory neurons depend on Schwann cells for survival. Schwann cells provide energy for axons dunng rapid firing or after injury. Dysregulated metabolism in Schwann cells can lead to the production of neurotoxic and axon degeneration. ABSTRACT Schwann cells are the glial cells in the peripheral nervous system responsible for the production ...
Rose Follis +2 more
wiley +1 more source
Sulfatides are found in brain as components of myelin, oligodendrocytes, and neurons but are also present in various visceral tissues. Metachromatic leukodystrophy (MLD) is an inherited lysosomal storage disorder caused by a deficiency of arylsulfatase A,
Mina Mirzaian +2 more
doaj +1 more source
TFEB regulates lysosomal proteostasis [PDF]
Loss-of-function diseases are often caused by destabilizing mutations that lead to protein misfolding and degradation. Modulating the innate protein homeostasis (proteostasis) capacity may lead to rescue of native folding of the mutated variants, thereby
Abrahamov +80 more
core +1 more source
Organoid‐based novel technology for antitumor drug screening
Organoids represent a cutting‐edge research model that closely mimics in vivo organ physiology, making them highly valuable for tumor immunotherapy drug screening. This review explores the integration of organoids with advanced technologies, such as gene editing, 3D bioprinting, and AI, highlighting novel fusion platforms and their applications.
Yu Su +3 more
wiley +1 more source
ABSTRACT Spasmodic dysphonia is a laryngeal dystonia that can present as adductor, abductor, or mixed types, with or without tremor. The etiology is not understood fully. Comprehensive evaluation is required to establish the diagnosis. Treatments include voice therapy, medications, botulinum toxin injection, laryngeal surgery, deep brain stimulation ...
Aaron J. Jaworek, Robert T. Sataloff
wiley +1 more source
Population carrier rates of pathogenic ARSA gene mutations: is metachromatic leukodystrophy underdiagnosed? [PDF]
BACKGROUND: Metachromatic leukodystrophy (MLD) is a severe neurometabolic disease caused mainly by deficiency of arylsulfatase A encoded by the ARSA gene.
Agnieszka Ługowska +4 more
doaj +1 more source
Rescue of splicing-mediated intron loss maximizes expression in lentiviral vectors containing the human ubiquitin C promoter. [PDF]
Lentiviral vectors almost universally use heterologous internal promoters to express transgenes. One of the most commonly used promoter fragments is a 1.2-kb sequence from the human ubiquitin C (UBC) gene, encompassing the promoter, some enhancers, first
Cooper, Aaron R +3 more
core +1 more source
ACE can modulate the CBL‐mediated K48 ubiquitination degradation of PSAP by altering its glycosylation levels in NP cells. As a result, NP cells secrete PSAP, which interacts with GPR37 on macrophage surfaces, facilitating their polarization toward the M2 phenotype. These M2 macrophages subsequently secrete TGFβ, which exerts feedback effects on the NP
Youfeng Guo +6 more
wiley +1 more source

