Gelpi Ellen, Visanji Naomi P, Hönigschnabl Selma, Reiner Angelika, Fischer Peter, Lang Anthony E, Budka Herbert, Kovacs Gabor G
Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, Vienna, Austria.
Neurological Tissue Bank of the Biobanc-Hospital Clinic-IDIBAPS, Barcelona, Spain.
Free Neuropathol. 2020 Feb 12;1:6. doi: 10.17879/freeneuropathology-2020-2616. eCollection 2020 Jan.
Deposition of alpha-synuclein in the brain is a hallmark of Lewy body disorders. Alpha-synuclein has been considered to show prion-like properties. Prion diseases can be transmitted by the transplantation of cadaveric dura mater causing iatrogenic Creutzfeldt-Jakob disease. Recent observations of amyloid-β deposition in dural grafts support the seeding properties of amyloid-β. Here we assessed the presence of alpha-synuclein in dura mater samples as a potential transmissible seed source. We immunostained 32 dura mater samples; 16 cases with Lewy-body disorder (LBD) showing different pathology stages and 16 non-LBD cases for phosphorylated (Ser129) and disease-associated (5G4) alpha-synuclein. Disease-associated alpha-synuclein aggregates were identified in intradural nerve fibres and associated with a vessel in a single LBD-Braak stage 4 case. We conclude that alpha-synuclein is detectable, although rarely, in dura mater samples in patients with LBD. The risk of potential transmissibility of dural alpha-synuclein deserves assessment by complementary experimental studies.
α-突触核蛋白在大脑中的沉积是路易体疾病的一个标志。α-突触核蛋白被认为具有朊病毒样特性。朊病毒疾病可通过移植尸体硬脑膜传播,导致医源性克雅氏病。最近在硬脑膜移植物中观察到的淀粉样β蛋白沉积支持了淀粉样β蛋白的播种特性。在这里,我们评估了硬脑膜样本中α-突触核蛋白的存在,作为一种潜在的可传播种子来源。我们对32个硬脑膜样本进行了免疫染色;16例路易体疾病(LBD)病例,显示不同病理阶段,以及16例非LBD病例,检测磷酸化(Ser129)和疾病相关(5G4)α-突触核蛋白。在一例LBD-Braak 4期病例的硬脑膜内神经纤维中发现了与疾病相关的α-突触核蛋白聚集体,并与一条血管相关。我们得出结论,在LBD患者的硬脑膜样本中可以检测到α-突触核蛋白,尽管很少见。硬脑膜α-突触核蛋白潜在传播的风险值得通过补充实验研究进行评估。