期刊
MOVEMENT DISORDERS
卷 30, 期 8, 页码 1085-1089出版社
WILEY-BLACKWELL
DOI: 10.1002/mds.26278
关键词
glucocerebrosidase; lysosomes; Parkinson's disease; sphingolipids
资金
- Wellcome Trust/MRC [WT089698]
- MRC Centre of Excellence in Neurodegeneration [MR/L501499/1]
- Parkinson's UK [G-1104]
- NHIR
- MRC [MC_G1000735] Funding Source: UKRI
- Medical Research Council [MC_G1000735, MR/L501499/1] Funding Source: researchfish
- National Institute for Health Research [NF-SI-0611-10237] Funding Source: researchfish
- Parkinson's UK [G-1104] Funding Source: researchfish
BackgroundTo establish whether Parkinson's disease (PD) brains previously described to have decreased glucocerebrosidase activity exhibit accumulation of the lysosomal enzyme's substrate, glucosylceramide, or other changes in lipid composition. MethodsLipidomic analyses and cholesterol measurements were performed on the putamen (n=5-7) and cerebellum (n=7-14) of controls, Parkinson's disease brains with heterozygote GBA1 mutations (PD+GBA), or sporadic PD. ResultsTotal glucosylceramide levels were unchanged in both PD+GBA and sporadic PD brains when compared with controls. No changes in glucosylsphingosine (deacetylated glucosylceramide), sphingomyelin, gangliosides (GM2, GM3), or total cholesterol were observed in either putamen or cerebellum. ConclusionsThis study did not demonstrate glucocerebrosidase substrate accumulation in PD brains with heterozygote GBA1 mutations in areas of the brain with low -synuclein pathology. (c) 2015 The Authors. Movement Disorders published by Wiley Periodicals, Inc. on behalf of International Parkinson and Movement Disorder Society.
作者
我是这篇论文的作者
点击您的名字以认领此论文并将其添加到您的个人资料中。
推荐
暂无数据