Functions of 'A disintegrin and metalloproteases (ADAMs)'in the mammalian nervous system

HE Hsia, J Tüshaus, T Brummer, Y Zheng… - Cellular and Molecular …, 2019 - Springer
Abstract 'A disintegrin and metalloproteases'(ADAMs) are a family of transmembrane
proteins with diverse functions in multicellular organisms. About half of the ADAMs are active …

Cellular and molecular mechanisms of prion disease

CJ Sigurdson, JC Bartz, M Glatzel - Annual Review of Pathology …, 2019 - annualreviews.org
Prion diseases are rapidly progressive, incurable neurodegenerative disorders caused by
misfolded, aggregated proteins known as prions, which are uniquely infectious. Remarkably …

Ligands binding to the prion protein induce its proteolytic release with therapeutic potential in neurodegenerative proteinopathies

L Linsenmeier, B Mohammadi, M Shafiq, K Frontzek… - Science …, 2021 - science.org
The prion protein (PrPC) is a central player in neurodegenerative diseases, such as prion
diseases or Alzheimer's disease. In contrast to disease-promoting cell surface PrPC …

Prion protein: the molecule of many forms and faces

V Kovač, V Čurin Šerbec - International journal of molecular sciences, 2022 - mdpi.com
Cellular prion protein (PrPC) is a glycosylphosphatidylinositol (GPI)-anchored protein most
abundantly found in the outer membrane of neurons. Due to structural characteristics (a …

Prion protein glycans reduce intracerebral fibril formation and spongiosis in prion disease

AM Sevillano, P Aguilar-Calvo, TD Kurt… - The Journal of …, 2020 - Am Soc Clin Investig
Posttranslational modifications (PTMs) are common among proteins that aggregate in
neurodegenerative disease, yet how PTMs impact the aggregate conformation and disease …

GPI-anchor signal sequence influences PrPC sorting, shedding and signalling, and impacts on different pathomechanistic aspects of prion disease in mice

B Puig, HC Altmeppen, L Linsenmeier… - PLoS …, 2019 - journals.plos.org
The cellular prion protein (PrPC) is a cell surface glycoprotein attached to the membrane by
a glycosylphosphatidylinositol (GPI)-anchor and plays a critical role in transmissible …

Proteolysis of CD44 at the cell surface controls a downstream protease network

B Wöhner, W Li, S Hey, A Drobny, L Werny… - Frontiers in Molecular …, 2023 - frontiersin.org
The cell surface receptor cluster of differentiation 44 (CD44) is the main hyaluronan receptor
of the human body. At the cell surface, it can be proteolytically processed by different …

Muskelin coordinates PrPC lysosome versus exosome targeting and impacts prion disease progression

FF Heisler, Y Pechmann, I Wieser, HC Altmeppen… - Neuron, 2018 - cell.com
Cellular prion protein (PrP C) modulates cell adhesion and signaling in the brain.
Conversion to its infectious isoform causes neurodegeneration, including Creutzfeldt-Jakob …

[HTML][HTML] The prion protein and its ligands: Insights into structure-function relationships

M Shafiq, S Da Vela, L Amin, N Younas… - … et Biophysica Acta (BBA …, 2022 - Elsevier
The prion protein is a multifunctional protein that exists in at least two different folding states.
It is subject to diverse proteolytic processing steps that lead to prion protein fragments some …

Prion protein quantification in human cerebrospinal fluid as a tool for prion disease drug development

SM Vallabh, CK Nobuhara, F Llorens… - Proceedings of the …, 2019 - National Acad Sciences
Reduction of native prion protein (PrP) levels in the brain is an attractive strategy for the
treatment or prevention of human prion disease. Clinical development of any PrP-reducing …