Figure 7.
MAb RD3 detects not only intracellular neurofibrillary tangles (NFTs) at 3 stages (0, 1, 2) of maturation, but also ghost tangles (GT) in different stages of degradation. In stage 0 (pretangles), RD3-positive granular material appears in a portion of cytoplasm in neurons with normal morphology. In stage 1 (early tangles), immunoreactive material aggregates in small, rod-shaped bundles of fibrils. Large, flame-shaped, RD3-positive inclusions filling almost the entire cell cytoplasm correspond to stage 2 (mature tangles). GTs (extracellular NFTs) represent the end stage (stage 3) of neurofibrillary degeneration. They are strongly RD3-positive, loosely arranged clusters of extracellular bundles of abnormal fibers exceeding by 2 to 3 times the size of degraded neuron. In the lower row, 3-color immunostaining of tau protein (red; mAb Tau1), alternative splicing factor (ASF) (green, pAb Ab 38017), and nuclei (blue) in the frontal cortex of a 67-year-old DS subject does not show colocalization of ASF and NFTs.