Results 71 to 80 of about 1,083 (177)

Palmitoyl‐Protein Thioesterase 1 (PPT1) Protein, Linked to Neuronal Ceroid Lipofuscinosis 1, Is a Major Constituent of Ageing‐Related Human Neuronal Lipofuscin

Neuropathology and Applied Neurobiology, Volume 51, Issue 5, October 2025.
ABSTRACT Proteomics of laser‐dissected lipofuscin from aged, healthy brains reveals Palmitoyl‐Protein Thioesterase 1 (PPT1) and other CLN proteins as constituents. PPT1 is increasingly sequestered to lipofuscin during ageing. Protein sequestering into lipofuscin may contribute to physiological neuronal ageing.
Max Anstötz, Sarah Tschirner, Caroline May, Steffen Kösters, Christine Martin, Svenja Caspers, Eleonora Aronica, Hans Jürgen Bidmon, Katrin Marcus, Carsten Korth   +9 more
wiley   +1 more source

Molecular role of sulfated glycoprotein-1 (SGP-I/Prosaposin) in Sertoli cells [PDF]

, 1995
Sulfated Glycoprotein- 1 (SGP- 1) is a major polypeptide secreted by rat Sertoli cells. Sequence analysis revealed a 70% sequence similarity with human prosaposin and a 80% similarity with mouse prosaposin.
Morales, C.R., Igdoura, S., Zhao, Q., El-Alfy, M.   +3 more
core  

Cathepsin-mediated regulation of autophagy in saposin C deficiency [PDF]

Autophagy, 2013
Saposin C deficiency, a rare variant form of Gaucher disease, is due to mutations in the prosaposin gene (PSAP) affecting saposin C expression and/or function. We previously reported that saposin C mutations affecting one cysteine residue result in autophagy dysfunction.
Tatti M, Motta M, DI BARTOLOMEO, Sabrina, Cianfanelli V, Salvioli R.   +4 more
openaire   +2 more sources

Human Saposin B Ligand Binding and Presentation to α-Galactosidase A

, 2022
Saposins are helix bundle proteins which solubilize sphingolipids and present them to lysosomal hydrolases for catabolism. Saposin B (SapB) is an activator of globotriaosylceramide (Gb3) catabolism by α-galactosidase A (GLA).
Sawyer, Thomas K.
core   +1 more source

Target lysis by cholesterol extraction is a rate limiting step in the resolution of phagolysosomes

European Journal of Cell Biology
The ongoing phagocytic activity of macrophages necessitates an extraordinary capacity to digest and resolve incoming material. While the initial steps leading to the formation of a terminal phagolysosome are well studied, much less is known about the ...
Dante Barreda, Sergio Grinstein, Spencer A. Freeman   +2 more
doaj   +1 more source

Gaucher disease, state of the art and perspectives

Journal of Internal Medicine, Volume 298, Issue 3, Page 155-172, September 2025.
Abstract Knowledge about Gaucher disease (GD), considered a model for rare diseases, has considerably increased since its discovery. The pathophysiology of this lysosomal disorder is better known, and specific therapies that can control many aspects of the disease have been developed, particularly for the most common form, Type 1 GD.
Fabrice Camou, Marc G. Berger
wiley   +1 more source

Structural and Functional Studies of the Human Saposin Proteins

, 2012
The human saposins are four homologous activator proteins that are essential for the lysosomal degradation of sphingolipids (SLs) with small headgroups. They function in part to increase the solvent accessibility of these SLs to specific acid hydrolases
Popovic, Konstantin
core   +1 more source

How the diversity in digestion in carnivorous plants may have evolved

New Phytologist, Volume 247, Issue 6, Page 2581-2590, September 2025.
Summary Carnivorous plants secrete digestive enzymes for prey degradation. Although carnivorous plants have a polyphyletic origin and evolved several times independently, they surprisingly co‐opted similar digestive enzymes during convergent evolution.
Andrej Pavlovič
wiley   +1 more source

Interaction of saposins, acidic lipids, and glucosylceramidase.

Journal of Biological Chemistry, 1990
Activity of lysosomal glucosylceramidase is stimulated by two small glycoproteins, saposin A and C, which are, together with two other similar glycoproteins, derived from a single precursor protein. This enzyme is also stimulated by naturally occurring acidic lipids, such as phosphatidylserine and gangliosides. Using highly purified glucosylceramidase,
S, Morimoto, Y, Kishimoto, J, Tomich, S, Weiler, T, Ohashi, J A, Barranger, K A, Kretz, J S, O'Brien   +7 more
openaire   +2 more sources

Deficiency of Glucocerebrosidase Activity beyond Gaucher Disease: PSAP and LIMP-2 Dysfunctions [PDF]

Int J Mol Sci
Glucocerebrosidase (GCase) is a lysosomal enzyme that catalyzes the breakdown of glucosylceramide in the presence of its activator saposin C (SapC). SapC arises from the proteolytical cleavage of prosaposin (encoded by PSAP gene), which gives rise to ...
Pavan E, Peruzzo P, Cattarossi S, Bergamin N, Bordugo A, Sechi A, Scarpa M, Biasizzo J, Colucci F, Dardis A.   +9 more
europepmc   +3 more sources