| Infantile-onset symptomatic epilepsy syndrome caused by a homozygous loss-of-function mutation of GM3 synthase MA Simpson, H Cross, C Proukakis, DA Priestman, DCA Neville, ... Nature genetics 36 (11), 1225-1229, 2004 | 456 | 2004 |
| Glucose fatty acid interactions and the regulation of glucose disposal PJ Randle, DA Priestman, SC Mistry, A Halsall Journal of cellular biochemistry 55 (S1994A), 1-11, 1994 | 253 | 1994 |
| Mechanisms modifying glucose oxidation in diabetes mellitus PJ Randle, DA Priestman, S Mistry, A Halsall Diabetologia 37, S155-S161, 1994 | 213 | 1994 |
| Analysis of fluorescently labeled glycosphingolipid-derived oligosaccharides following ceramide glycanase digestion and anthranilic acid labeling DCA Neville, V Coquard, DA Priestman, DJM te Vruchte, DJ Sillence, ... Analytical biochemistry 331 (2), 275-282, 2004 | 206 | 2004 |
| Implications for invariant natural killer T cell ligands due to the restricted presence of isoglobotrihexosylceramide in mammals AO Speak, M Salio, DCA Neville, J Fontaine, DA Priestman, N Platt, ... Proceedings of the National Academy of Sciences 104 (14), 5971-5976, 2007 | 184 | 2007 |
| Heat shock protein–based therapy as a potential candidate for treating the sphingolipidoses T Kirkegaard, J Gray, DA Priestman, KL Wallom, J Atkins, OD Olsen, ... Science translational medicine 8 (355), 355ra118-355ra118, 2016 | 169 | 2016 |
| Fetal gene therapy for neurodegenerative disease of infants G Massaro, CNZ Mattar, AMS Wong, E Sirka, SMK Buckley, BR Herbert, ... Nature medicine 24 (9), 1317-1323, 2018 | 143 | 2018 |
| Inhibition of substrate synthesis as a strategy for glycolipid lysosomal storage disease therapy FM Platt, M Jeyakumar, U Andersson, DA Priestman, RA Dwek, ... Journal of inherited metabolic disease 24 (2), 275-290, 2001 | 140 | 2001 |
| Mutations in B4GALNT1 (GM2 synthase) underlie a new disorder of ganglioside biosynthesis GV Harlalka, A Lehman, B Chioza, EL Baple, R Maroofian, H Cross, ... Brain 136 (12), 3618-3624, 2013 | 133 | 2013 |
| Reduced sphingolipid hydrolase activities, substrate accumulation and ganglioside decline in Parkinson’s disease M Huebecker, EB Moloney, AC van der Spoel, DA Priestman, O Isacson, ... Molecular neurodegeneration 14, 1-21, 2019 | 116 | 2019 |
| A novel approach to analyze lysosomal dysfunctions through subcellular proteomics and lipidomics: the case of NPC1 deficiency AK Tharkeshwar, J Trekker, W Vermeire, J Pauwels, R Sannerud, ... Scientific reports 7 (1), 41408, 2017 | 112 | 2017 |
| Amyotrophic lateral sclerosis and denervation alter sphingolipids and up-regulate glucosylceramide synthase A Henriques, V Croixmarie, DA Priestman, A Rosenbohm, S Dirrig-Grosch, ... Human molecular genetics 24 (25), 7390-7405, 2015 | 96 | 2015 |
| Increased glycosphingolipid levels in serum and aortae of apolipoprotein E gene knockout mice B Garner, DA Priestman, R Stocker, DJ Harvey, TD Butters, FM Platt Journal of lipid research 43 (2), 205-214, 2002 | 81 | 2002 |
| Glycosphingolipid levels and glucocerebrosidase activity are altered in normal aging of the mouse brain PJ Hallett, M Huebecker, OR Brekk, EB Moloney, EM Rocha, ... Neurobiology of aging 67, 189-200, 2018 | 79 | 2018 |
| Presence in human cells and tissues of two prolidases and their alteration in prolidase deficiency J Butterworth, DA Priestman Journal of inherited metabolic disease 8 (4), 193-197, 1985 | 64 | 1985 |
| Neuraminidases 3 and 4 regulate neuronal function by catabolizing brain gangliosides X Pan, C De Britto Pará De Aragão, JP Velasco‐Martin, DA Priestman, ... The FASEB Journal 31 (8), 3467-3483, 2017 | 62 | 2017 |
| Ionization and fragmentation of neutral and acidic glycosphingolipids with a Q-TOF mass spectrometer fitted with a MALDI ion source V Hunnam, DJ Harvey, DA Priestman, RH Bateman, RS Bordoli, ... Journal of the American Society for Mass Spectrometry 12 (11), 1220-1225, 2001 | 57 | 2001 |
| Studies of the long-term regulation of hepatic pyruvate dehydrogenase kinase CM SUGDEN, GDL FRYER, AK ORFALI, AD PRIESTMAN, E DONALD, ... Biochemical Journal 329 (1), 89-94, 1998 | 50 | 1998 |
| Brain pathology in mucopolysaccharidoses (MPS) patients with neurological forms GM Viana, DA Priestman, FM Platt, S Khan, S Tomatsu, AV Pshezhetsky Journal of clinical medicine 9 (2), 396, 2020 | 47 | 2020 |
| Fasting and decreased B cell sensitivity: important role for fatty acid-induced inhibition of PDH activity YP Zhou, DA Priestman, PJ Randle, VE Grill American Journal of Physiology-Endocrinology and Metabolism 270 (6), E988-E994, 1996 | 47 | 1996 |
raymond dwekoxford glycobiology instituteEmail verificata su exeter.ox.ac.uk
