Brain Pathology in Mucopolysaccharidoses (MPS) Patients with Neurological Forms

by Gustavo M. Viana, David A. Priestman, Frances M. Platt, Shaukat Khan, Shunji Tomatsu, Alexey V. Pshezhetsky
Published 2020-02-01

Increased glycosphingolipid levels in serum and aortae of apolipoprotein E gene knockout mice

by Brett Garner, David A. Priestman, Roland Stocker, David J. Harvey, Terry D. Butters, Frances M. Platt
Published 2002-02-01

Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease

by Mylene Huebecker, Elizabeth B. Moloney, Aarnoud C. van der Spoel, David A. Priestman, Ole Isacson, Penelope J. Hallett, Frances M. Platt
Published 2019-11-01

Correction to: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease

by Mylene Huebecker, Elizabeth B. Moloney, Aarnoud C. van der Spoel, David A. Priestman, Ole Isacson, Penelope J. Hallett, Frances M. Platt
Published 2020-01-01

Inhibition of β-Glucocerebrosidase Activity Preserves Motor Unit Integrity in a Mouse Model of Amyotrophic Lateral Sclerosis

by Alexandre Henriques, Mylene Huebecker, Hélène Blasco, Céline Keime, Christian R. Andres, Philippe Corcia, David A. Priestman, Frances M. Platt, Michael Spedding, Jean-Philippe Loeffler
Published 2017-07-01

Altered Expression of Ganglioside Metabolizing Enzymes Results in GM3 Ganglioside Accumulation in Cerebellar Cells of a Mouse Model of Juvenile Neuronal Ceroid Lipofuscinosis

by Aleksandra Somogyi, Anton Petcherski, Benedikt Beckert, Mylene Huebecker, David A. Priestman, Antje Banning, Susan L. Cotman, Frances M. Platt, Mika O. Ruonala, Ritva Tikkanen
Published 2018-02-01

A modified density gradient proteomic-based method to analyze endolysosomal proteins in cardiac tissue

by Thamali Ayagama, Samuel J. Bose, Rebecca A. Capel, David A. Priestman, Georgina Berridge, Roman Fischer, Antony Galione, Frances M. Platt, Holger Kramer, Rebecca A.B. Burton
Published 2021-09-01

Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity

by Anyelo Durán, Boris Rebolledo-Jaramillo, Valeria Olguin, Marcelo Rojas-Herrera, Macarena Las Heras, Juan F. Calderón, Silvana Zanlungo, David A. Priestman, Frances M. Platt, Andrés D. Klein
Published 2021-12-01

Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity

by Oeystein R. Brekk, Joanna A. Korecka, Cecile C. Crapart, Mylene Huebecker, Zachary K. MacBain, Sara Ann Rosenthal, Miguel Sena-Esteves, David A. Priestman, Frances M. Platt, Ole Isacson, Penelope J. Hallett
Published 2020-08-01

TLR9-mediated dendritic cell activation uncovers mammalian ganglioside species with specific ceramide backbones that activate invariant natural killer T cells.

by Christophe Paget, Shenglou Deng, Daphnée Soulard, David A Priestman, Silvia Speca, Johanna von Gerichten, Anneliese O Speak, Ashish Saroha, Yael Pewzner-Jung, Anthony H Futerman, Thierry Mallevaey, Christelle Faveeuw, Xiaobo Gu, Frances M Platt, Roger Sandhoff, François Trottein
Published 2019-03-01

Molecular basis for a new bovine model of Niemann-Pick type C disease.

by Shernae A Woolley, Emily R Tsimnadis, Cor Lenghaus, Peter J Healy, Keith Walker, Andrew Morton, Mehar S Khatkar, Annette Elliott, Ecem Kaya, Clarisse Hoerner, David A Priestman, Dawn Shepherd, Frances M Platt, Ben T Porebski, Cali E Willet, Brendon A O'Rourke, Imke Tammen
Published 2020-01-01