Results 201 to 210 of about 41,053 (230)

Lipofuscin accumulation in aging and neurodegeneration: a potential "timebomb" overlooked in Alzheimer's disease. [PDF]

Transl Neurodegener
Dougnon G, Matsui H.
europepmc   +1 more source

From Molecule to Meaning: Neuronopathic Biomarkers and Clinical Relevance in GM1. [PDF]

J Inherit Metab Dis
Casazza K, Giugliani R, Regier DS, Jarnes J.   +3 more
europepmc   +1 more source

Hepatic Steatosis: A Presentation of Cholesteryl Ester Storage Disease. [PDF]

ACG Case Rep J
Lee A, Raasikh T, Bangaru S, Yano S, Chopra S, Saito T.   +5 more
europepmc   +1 more source

Genetic Modeling of Lysosomal Storage Disorders (LSDs) in the Brain-Midgut Axis of <i>Drosophila melanogaster</i> During Aging. [PDF]

Cells
Markaki SP, Kiose NM, Charitopoulou ZA, Kougioumtzoglou S, Velentzas AD, Stravopodis DJ.   +5 more
europepmc   +1 more source

Enzyme replacement therapy during pregnancy and breastfeeding in late-onset Pompe disease. [PDF]

Int Breastfeed J
Bachmann M, Kasper D, Scholl-Bürgi S, Margreitter J, Höller A, Löscher W, Karall D, Zöggeler T.   +7 more
europepmc   +1 more source

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Lysosomal Glycosphingolipid Storage Diseases

Annual Review of Biochemistry, 2019
Glycosphingolipids are cell-type-specific components of the outer leaflet of mammalian plasma membranes. Gangliosides, sialic acid–containing glycosphingolipids, are especially enriched on neuronal surfaces. As amphi-philic molecules, they comprise a hydrophilic oligosaccharide chain attached to a hydrophobic membrane anchor, ceramide.
Bernadette, Breiden, Konrad, Sandhoff
openaire   +2 more sources

Lysosomal storage diseases

Lysosomal storage disorders (LSDs) are a group of inherited metabolic diseases caused by dysfunction of the lysosomal system, with subsequent progressive accumulation of macromolecules, activation of inflammatory response, and cell death. Neurologic damage is almost always present, and it is usually degenerative.
Alessandro P, Burlina, Renzo, Manara, Daniela, Gueraldi   +2 more
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LYSOSOMAL STORAGE DISEASES

Neuropathology and Applied Neurobiology, 1978
The majority of lysosomal storage diseases affect the central nervous system. Those that reflect a primary lysosomal disorder are associated with genetically determined deficiencies of specific lysosomal enzymes and storage of the relevant substrate. Autofluorescent lipopigments accumulate in the ceroid‐lipofuscinoses, a heterogeneous group of diseases
openaire   +2 more sources