Trojanowski J Q, Schmidt M L, Otvos L, Gur R C, Gur R E, Hurtig H, Lee V M
Department of Pathology and Laboratory Medicine, University of Pennsylvania School of Medicine, Philadelphia 19104-4283.
Ann Med. 1989;21(2):113-6. doi: 10.3109/07853898909149196.
Here we review our recent "epitope analyses" of a few of the fibrous intraneuronal inclusions that are distinctive hallmarks of human neurodenerative conditions using a large library of monoclonal antibodies (MAbs) raised to normal neuronal cytoskeletal proteins. Analyses of the low (NF-L), middle (NF-M), and high (NF-M), and high (NF-H) molecular weight neurofilament (NF) proteins with greater than 500 MAbs enumerated epitopes shared by NF proteins and the intraneuronal neurofibrillary tangles (NFTs) that occur in the hippocampus and brainstem of Alzheimer's disease (AD) subjects. We identified the NF-H multi-phosphorylation repeat domain, i.e. repeats of Lys-Ser-Pro-X (where X is a small uncharged amino acid and Ser acts as a phosphate acceptor), as the determinant recognized by 15/16 MAbs that detected NFTs in sections of AD hippocampus, and 11 of the same 16 MAbs recognised NF-M multi-phosphorylation repeats. Further, the antigen binding regions of these MAbs were shown to comprise 13 separate classes based on their differential binding to 12 synthetic peptides derived from the NF-H and NF-M multi-phosphorylation sites, NF subunits of 10 diverse mammalian and sub-mammalian species, and normal human tau (tau). None of these anti-NF MAbs recognized NFTs in the brainstem of subjects with progressive supranuclear palsy (PSP), but NFTs in AD brainstem sections were reactive with five of these MAbs. Both PSP and AD brainstem NFTs were recognized by MAbs specific for tau and paired helical filament antigens.(ABSTRACT TRUNCATED AT 400 WORDS)
在此,我们回顾了我们最近利用针对正常神经元细胞骨架蛋白产生的大量单克隆抗体(MAb)文库,对一些纤维状神经元内包涵体进行的“表位分析”。这些包涵体是人类神经退行性疾病的独特标志。我们用500多种MAb对低分子量(NF-L)、中分子量(NF-M)和高分子量(NF-H)神经丝(NF)蛋白进行分析,列举了NF蛋白与阿尔茨海默病(AD)患者海马体和脑干中出现的神经元内神经原纤维缠结(NFT)共有的表位。我们确定NF-H多磷酸化重复结构域,即赖氨酸-丝氨酸-脯氨酸-X(其中X是一个不带电荷的小氨基酸,丝氨酸作为磷酸受体)的重复序列,是15/16种在AD海马体切片中检测到NFT的MAb所识别的决定簇,并且这16种MAb中有11种识别NF-M多磷酸化重复序列。此外,根据这些MAb与12种源自NF-H和NF-M多磷酸化位点、10种不同哺乳动物和亚哺乳动物物种的NF亚基以及正常人tau(tau)的合成肽的差异结合,其抗原结合区域被证明包含13个不同类别。这些抗NF MAb均未识别进行性核上性麻痹(PSP)患者脑干中的NFT,但AD脑干切片中的NFT与其中5种MAb发生反应。PSP和AD脑干NFT均被针对tau和双螺旋丝抗原的MAb识别。(摘要截断于400字)