David A. Priestman
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View article: Plasma membrane remodeling in GM2 gangliosidoses drives synaptic dysfunction
Plasma membrane remodeling in GM2 gangliosidoses drives synaptic dysfunction Open
Glycosphingolipids (GSL) are important bioactive membrane components. GSLs containing sialic acids, known as gangliosides, are highly abundant in the brain and diseases of ganglioside metabolism cause severe early-onset neurodegeneration. …
View article: Plasma Membrane Remodelling in GM2 Gangliosidoses Drives Synaptic Dysfunction
Plasma Membrane Remodelling in GM2 Gangliosidoses Drives Synaptic Dysfunction Open
Glycosphingolipids (GSL) are important bioactive components of cellular membranes. Complex GSLs, containing sialic acid residues are known as gangliosides and are highly abundant in the brain. Diseases of ganglioside metabolism often resul…
View article: Analysis of glycosphingolipids from cell lines v1
Analysis of glycosphingolipids from cell lines v1 Open
Interest in the role of cellular glycosphingolipids (GSLs) in health and disease led to us developing a sensitive method to analyse the full complement of GSL structures present in mammalian cells, fluids and tissues. The original qualitat…
View article: Compartmentalisation proteomics revealed endolysosomal protein network changes in a goat model of atrial fibrillation
Compartmentalisation proteomics revealed endolysosomal protein network changes in a goat model of atrial fibrillation Open
Summary Endolysosomes (EL) are known for their role in regulating both intracellular trafficking and proteostasis. EL help facilitate elimination of damaged membrane and cytosolic proteins, protein aggregates, membranous organelles and als…
View article: A Mouse Systems Genetics Approach Reveals Common and Uncommon Genetic Modifiers of Hepatic Lysosomal Enzyme Activities and Glycosphingolipids
A Mouse Systems Genetics Approach Reveals Common and Uncommon Genetic Modifiers of Hepatic Lysosomal Enzyme Activities and Glycosphingolipids Open
Identification of genetic modulators of lysosomal enzyme activities and glycosphingolipids (GSLs) may facilitate the development of therapeutics for diseases in which they participate, including Lysosomal Storage Disorders (LSDs). To this …
View article: Glb1 knockout mouse model shares natural history with type II GM1 gangliosidosis patients
Glb1 knockout mouse model shares natural history with type II GM1 gangliosidosis patients Open
View article: A mouse systems genetics approach reveals common and uncommon genetic modifiers of hepatic lysosomal enzyme activities and glycosphingolipids
A mouse systems genetics approach reveals common and uncommon genetic modifiers of hepatic lysosomal enzyme activities and glycosphingolipids Open
Identification of genetic modulators of lysosomal enzyme activities and glycosphingolipids (GSLs) may facilitate the development of therapeutics for diseases in which they participate, including Lysosomal Storage Disorders (LSDs). To this …
View article: Analysis of glycosphingolipids from human cerebrospinal fluid v1
Analysis of glycosphingolipids from human cerebrospinal fluid v1 Open
Interest in the role of cellular glycosphingolipids (GSLs) in health and disease led to us developing a sensitive method to analyse the full complement of GSL structures present in mammalian cells, fluids and tissues. The original qualitat…
View article: Analysis of glycosphingolipids from animal tissues v1
Analysis of glycosphingolipids from animal tissues v1 Open
Interest in the role of cellular glycosphingolipids (GSLs) in health and disease led to us developing a sensitive method to analyse the full complement of GSL structures present in mammalian cells, fluids and tissues. The original qualitat…
View article: Invariant NKT cells metabolically adapt to the acute myeloid leukaemia environment
Invariant NKT cells metabolically adapt to the acute myeloid leukaemia environment Open
View article: Glycosphingolipid Changes in Plasma in Parkinson's Disease Independent of Glucosylceramide Levels
Glycosphingolipid Changes in Plasma in Parkinson's Disease Independent of Glucosylceramide Levels Open
Background Alteration in glycosphingolipids (GSLs) in Parkinson's disease (PD) still needs to be determined. Objectives We evaluated if PD subjects show abnormal GSLs levels compared to healthy controls (HC) and if GSLs correlate with clin…
View article: Glucosamine amends CNS pathology in mucopolysaccharidosis IIIC mouse expressing misfolded HGSNAT
Glucosamine amends CNS pathology in mucopolysaccharidosis IIIC mouse expressing misfolded HGSNAT Open
The majority of mucopolysaccharidosis IIIC (MPS IIIC) patients have missense variants causing misfolding of heparan sulfate acetyl-CoA:α-glucosaminide N-acetyltransferase (HGSNAT), which are potentially treatable with pharmacological chape…
View article: Glycosphingolipid metabolism and its role in ageing and Parkinson’s disease
Glycosphingolipid metabolism and its role in ageing and Parkinson’s disease Open
It is well established that lysosomal glucocerebrosidase gene ( GBA ) variants are a risk factor for Parkinson’s disease (PD), with increasing evidence suggesting a loss of function mechanism. One question raised by this genetic associatio…
View article: Correction: Molecular basis for a new bovine model of Niemann-Pick type C disease
Correction: Molecular basis for a new bovine model of Niemann-Pick type C disease Open
[This corrects the article DOI: 10.1371/journal.pone.0238697.].
View article: Oral Glucosamine Ameliorates Aggravated Neurological Phenotype in Mucopolysaccharidosis III Type C Mouse Model Expressing Misfolded HGSNAT Variant
Oral Glucosamine Ameliorates Aggravated Neurological Phenotype in Mucopolysaccharidosis III Type C Mouse Model Expressing Misfolded HGSNAT Variant Open
Objective Over 55% of mucopolysaccharidosis IIIC (MPS IIIC) patients have at least one allelic missense variant responsible for misfolding of heparan sulfate acetyl-CoA: α -glucosaminide N- acetyltransferase (HGSNAT). These variants are po…
View article: Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity
Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity Open
View article: A modified density gradient proteomic-based method to analyze endolysosomal proteins in cardiac tissue
A modified density gradient proteomic-based method to analyze endolysosomal proteins in cardiac tissue Open
View article: An iPSC model of hereditary sensory neuropathy-1 reveals L-serine-responsive deficits in neuronal ganglioside composition and axoglial interactions
An iPSC model of hereditary sensory neuropathy-1 reveals L-serine-responsive deficits in neuronal ganglioside composition and axoglial interactions Open
Hereditary sensory neuropathy type 1 (HSN1) is caused by mutations in the SPTLC1 or SPTLC2 sub-units of the enzyme serine palmitoyltransferase, resulting in the production of toxic 1-deoxysphingolipid bases (DSBs). We used in…
View article: Analysis of glycosphingolipids from human plasma v1
Analysis of glycosphingolipids from human plasma v1 Open
Interest in the role of cellular glycosphingolipids (GSLs) in health and disease led to us developing a sensitive method to analyse the full complement of GSL structures present in mammalian cells, fluids and tissues. The original qualitat…
View article: Cardiac Atrial Compartmentalisation Proteomics: A Modified Density Gradient Method to Analyse Endo-lysosomal Proteins
Cardiac Atrial Compartmentalisation Proteomics: A Modified Density Gradient Method to Analyse Endo-lysosomal Proteins Open
Summary The importance of lysosomes in cardiac physiology and pathology are well established, and evidence for roles in calcium signalling are emerging. We describe a label-free proteomics method suitable for small cardiac tissue biopsies …
View article: Molecular basis for a new bovine model of Niemann-Pick type C disease
Molecular basis for a new bovine model of Niemann-Pick type C disease Open
Niemann-Pick type C disease is a lysosomal storage disease affecting primarily the nervous system that results in premature death. Here we present the first report and investigation of Niemann-Pick type C disease in Australian Angus/Angus-…
View article: Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity
Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity Open
View article: Brain Pathology in Mucopolysaccharidoses (MPS) Patients with Neurological Forms
Brain Pathology in Mucopolysaccharidoses (MPS) Patients with Neurological Forms Open
Mucopolysaccharidoses (MPS) are the group of lysosomal storage disorders caused by deficiencies of enzymes involved in the stepwise degradation of glycosaminoglycans. To identify brain pathology common for neurological MPS, we conducted a …
View article: Brain pathology in MPS patients with neurological forms
Brain pathology in MPS patients with neurological forms Open
View article: Correction to: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease
Correction to: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease Open
The original article [1] contains an error in the y-axes of Fig. 8’s sub-figures whereby ‘CSF’ is mistakenly mentioned instead of ‘serum’.
View article: Systemic AAV9 gene therapy using the synapsin I promoter rescues a mouse model of neuronopathic Gaucher disease but with limited cross-correction potential to astrocytes
Systemic AAV9 gene therapy using the synapsin I promoter rescues a mouse model of neuronopathic Gaucher disease but with limited cross-correction potential to astrocytes Open
Gaucher disease is caused by mutations in the GBA gene, which encodes for the lysosomal enzyme β-glucocerebrosidase (GCase), resulting in the accumulation of storage material in visceral organs and in some cases the brain of affected patie…
View article: Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease
Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease Open
View article: Sterile activation of invariant natural killer T cells by ER-stressed antigen-presenting cells
Sterile activation of invariant natural killer T cells by ER-stressed antigen-presenting cells Open
Significance While there is a clear understanding of how invariant NKT (iNKT) cells are activated in foreign infection, it remains unclear how they are activated during sterile inflammation, including cancer, where they have a well-defined…
View article: TLR9-mediated dendritic cell activation uncovers mammalian ganglioside species with specific ceramide backbones that activate invariant natural killer T cells
TLR9-mediated dendritic cell activation uncovers mammalian ganglioside species with specific ceramide backbones that activate invariant natural killer T cells Open
CD1d-restricted invariant natural killer T (iNKT) cells represent a heterogeneous population of lipid-reactive T cells that are involved in many immune responses, mediated through T-cell receptor (TCR)-dependent and/or independent activati…
View article: MOESM3 of Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease
MOESM3 of Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease Open
Additional file 3. Statistics.