Mitochondrial ATP synthase subunit c storage in the ceroid‐lipofuscinoses (Batten disease) DN Palmer, IM Fearnley, JE Walker, NA Hall, BD Lake, LS Wolfe, M Haltia, ... American journal of medical genetics 42 (4), 561-567, 1992 | 418 | 1992 |
Storage of saposins A and D in infantile neuronal ceroid-lipofuscinosis J Tyynelä, DN Palmer, M Baumann, M Haltia FEBS letters 330 (1), 8-12, 1993 | 228 | 1993 |
Ovine ceroid lipofuscinosis: the major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence DN Palmer, RD Martinus, SM Cooper, GG Midwinter, JC Reid, RD Jolly Journal of Biological Chemistry 264 (10), 5736-5740, 1989 | 213 | 1989 |
Mutational analysis of the defective protease in classic late-infantile neuronal ceroid lipofuscinosis, a neurodegenerative lysosomal storage disorder DE Sleat, RM Gin, I Sohar, K Wisniewski, S Sklower-Brooks, RK Pullarkat, ... The American Journal of Human Genetics 64 (6), 1511-1523, 1999 | 198 | 1999 |
The sequence of the major protein stored in ovine ceroid lipofuscinosis is identical with that of the dicyclohexylcarbodiimide-reactive proteolipid of mitochondrial ATP synthase IM Fearnley, JE Walker, RD Martinus, RD Jolly, KB Kirkland, GJ Shaw, ... Biochemical journal 268 (3), 751-758, 1990 | 166 | 1990 |
Ceroid lipofuscinosis in sheep. II. The major component of the lipopigment in liver, kidney, pancreas, and brain is low molecular weight protein. DN Palmer, G Barns, DR Husbands, RD Jolly Journal of Biological Chemistry 261 (4), 1773-1777, 1986 | 148 | 1986 |
Glial activation spreads from specific cerebral foci and precedes neurodegeneration in presymptomatic ovine neuronal ceroid lipofuscinosis (CLN6) MJ Oswald, DN Palmer, GW Kay, SJA Shemilt, P Rezaie, JD Cooper Neurobiology of disease 20 (1), 49-63, 2005 | 138 | 2005 |
NCL disease mechanisms DN Palmer, LA Barry, J Tyynelä, JD Cooper Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1832 (11 …, 2013 | 133 | 2013 |
Lysosomal storage of the DCCD reactive proteolipid subunit of mitochondrial ATP synthase in human and ovine ceroid lipofuscinoses. DN Palmer, IM Fearnley, SM Medd, JE Walker, RD Martinus, SL Bayliss, ... Advances in experimental medicine and biology 266, 211-22; discussion 223, 1989 | 120 | 1989 |
Defective endoplasmic reticulum-resident membrane protein CLN6 affects lysosomal degradation of endocytosed arylsulfatase A C Heine, B Koch, S Storch, A Kohlschutter, DN Palmer, T Braulke Journal of Biological Chemistry 279 (21), 22347-22352, 2004 | 118 | 2004 |
Ligand partitioning into membranes: its significance in determining Km and Ks values for cytochrome P-450 and other membrane bound receptors and enzymes G Parry, DN Palmer, DJ Williams FEBS letters 67 (2), 123-129, 1976 | 113 | 1976 |
Separation of some neutral lipids by normal-phase high-performance liquid chromatography on a cyanopropyl column: ubiquinone, dolichol, and cholesterol levels in sheep liver DN Palmer, MA Anderson, RD Jolly Analytical Biochemistry 140 (2), 315-319, 1984 | 108 | 1984 |
Ceroid lipofuscinosis in sheep. I. Bis (monoacylglycero) phosphate, dolichol, ubiquinone, phospholipids, fatty acids, and fluorescence in liver lipopigment lipids. DN Palmer, DR Husbands, PJ Winter, JW Blunt, RD Jolly Journal of Biological Chemistry 261 (4), 1766-1772, 1986 | 100 | 1986 |
A missense mutation (c. 184C> T) in ovine CLN6 causes neuronal ceroid lipofuscinosis in Merino sheep whereas affected South Hampshire sheep have reduced levels of CLN6 mRNA I Tammen, PJ Houweling, T Frugier, NL Mitchell, GW Kay, JAL Cavanagh, ... Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease 1762 (10 …, 2006 | 93 | 2006 |
Sheep and other animals with ceroid‐lipofuscinoses: Their relevance to Batten disease RD Jolly, RD Martinus, DN Palmer American journal of medical genetics 42 (4), 609-614, 1992 | 88 | 1992 |
A new large animal model of CLN5 neuronal ceroid lipofuscinosis in Borderdale sheep is caused by a nucleotide substitution at a consensus splice site (c. 571+ 1G>>> A) leading … T Frugier, NL Mitchell, I Tammen, PJ Houweling, DG Arthur, GW Kay, ... Neurobiology of disease 29 (2), 306-315, 2008 | 87 | 2008 |
Different patterns of hydrophobic protein storage in different forms of neuronal ceroid lipofuscinosis (NCL, Batten disease) DN Palmer, RD Jolly, HC Van Mil, J Tyynelä, VJ Westlake Neuropediatrics 28 (01), 45-48, 1997 | 84 | 1997 |
Ceroid–lipofuscinosis (Batten's disease): pathogenesis and sequential neuropathological changes in the ovine model RD Jolly, A Shimada, I Dopfmer, PM Slack, MJ Birtles, DN Palmer Neuropathology and applied Neurobiology 15 (4), 371-383, 1989 | 84 | 1989 |
Canine ceroid‐lipofuscinoses: A review and classification RD Jolly, DN Palmer, VP Studdert, RH Sutton, WR Kelly, N Koppang, ... Journal of Small Animal Practice 35 (6), 299-306, 1994 | 83 | 1994 |
Accumulation of the adenosine triphosphate synthase subunit C in the mnd mutant mouse. A model for neuronal ceroid lipofuscinosis. CA Pardo, BA Rabin, DN Palmer, DL Price The American journal of pathology 144 (4), 829, 1994 | 82 | 1994 |