Results 31 to 40 of about 1,105,094 (212)

Next-Generation Sequencing Analysis Reveals Novel Pathogenic Variants in Four Chinese Siblings With Late-Infantile Neuronal Ceroid Lipofuscinosis

open access: yesFrontiers in Genetics, 2019
Neuronal Ceroid Lipofuscinoses (NCLs) are progressive degenerative diseases mainly affect brain and retina. They are characterized by accumulation of autofluorescent storage material, mitochondrial ATPase subunit C, or sphingolipid activator proteins A ...
Xiao-Tun Ren   +8 more
doaj   +1 more source

Regulation of Mitogen-Activated Protein Kinases by Sphingolipid Products in Oligodendrocytes [PDF]

open access: yesThe Journal of Neuroscience, 1999
Sphingolipid products such as ceramide (cer), sphingosine (sph), and sphingosine-1-phosphate (SPP) are implicated in the regulation of cell growth and apoptosis. We have recently shown that cer, sph, and SPP differentially modulate ionic events in cultured oligodendrocytes (OLGs).
H, Hida   +3 more
openaire   +2 more sources

Processing of sphingolipid activator proteins and the topology of lysosomal digestion.

open access: yesActa Biochimica Polonica, 1998
Plasma membrane derived glycosphingolipids (GSLs) destined for digestion are internalized through the endocytic pathway and delivered to the lysosomes. There, GSLs are degraded by the action of exohydrolases, which are supported, in the case of GSLs with
K. Sandhoff, T. Kolter
semanticscholar   +1 more source

CPTP: A sphingolipid transfer protein that regulates autophagy and inflammasome activation [PDF]

open access: yesAutophagy, 2018
The macroautophagy/autophagy and inflammasome pathways are linked through their roles in innate immunity and chronic inflammatory disease. Ceramide-1-phosphate (C1P) is a bioactive sphingolipid that regulates pro-inflammatory eicosanoid production. Whether C1P also regulates autophagy and inflammasome assembly/activation is not known. Here we show that
Shrawan Kumar Mishra   +5 more
openaire   +3 more sources

Lysosomal proteolysis of prosaposin, the precursor of saposins (sphingolipid activator proteins): its mechanism and inhibition by ganglioside.

open access: yesArchives of Biochemistry and Biophysics, 1997
Saposins A, B, C, and D, which are required for the enzymatic hydrolysis of sphingolipids by specific lysosomal hydrolases, are produced by proteolytic processing of their common precursor protein, prosaposin.
M. Hiraiwa   +5 more
semanticscholar   +1 more source

Do Sphingolipids Control Viral Membrane Protein Activity? [PDF]

open access: yesBiophysical Journal, 2011
The membrane-behaviour of the matrix (M) protein from respiratory syncytical virus, a prototypical enveloped virus, has been characterised by surface techniques (tensiometry, Brewster angle microscopy and atomic force microscopy) and experiments using lipid bilayers (FRET, light microscopy).
Sanderson, John M.   +3 more
openaire   +1 more source

Phosphatidylinositol 4‐kinase as a target of pathogens—friend or foe?

open access: yesFEBS Letters, EarlyView.
This graphical summary illustrates the roles of phosphatidylinositol 4‐kinases (PI4Ks). PI4Ks regulate key cellular processes and can be hijacked by pathogens, such as viruses, bacteria and parasites, to support their intracellular replication. Their dual role as essential host enzymes and pathogen cofactors makes them promising drug targets.
Ana C. Mendes   +3 more
wiley   +1 more source

Microbiome−host proteostasis crosstalk—An emerging perspective on mechanisms and interventions toward healthy longevity

open access: yesFEBS Letters, EarlyView.
Proteostasis and the gut microbiota play a key role in shaping host physiology. Microbiota‐derived metabolites, vitamins, and RNA modulate host proteostasis. Findings from model systems, including C. elegans, indicate microbes can either stabilize or disrupt host proteostasis.
Abhishek Anil Dubey, Maria Ermolaeva
wiley   +1 more source

Aging Is a Key Driver for Adult Acute Myeloid Leukemia

open access: yesAging and Cancer, EarlyView.
Acute myeloid leukemia (AML) is a classical age‐related hematologic malignancy, and a key driver of AML is aging, which profoundly regulates intrinsic factors such as genomic instability, epigenetic reprogramming, and metabolic dysregulation, and alters bone marrow microenvironment.
Rong Yin, Haojian Zhang
wiley   +1 more source

Microfluidic Nano‐Assembly of Red‐Blood‐Cell (RBC) Lipids and Components for Engineering Extracellular Vesicles

open access: yesAdvanced Healthcare Materials, EarlyView.
Engineered red blood cell‐derived extracellular vesicles (eRBCEVs) are synthesized via controlled microfluidic assembly from native RBC lipids, enabling tunable encapsulation of proteins, nucleic acids, nanoparticles, and viral vectors. The platform demonstrates reproducible nanoscale architecture, preserved membrane composition, and functional cargo ...
Chiranth K. Nagaraj   +23 more
wiley   +1 more source

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