caa a22 4500 465767958 CHVBK 20180323111920.0 cr unu---uuuuu 170327e19900701xx s 000 0 eng 10.1007/BF00294653 doi (NATIONALLICENCE)springer-10.1007/BF00294653 Ubiquitin and phosphorylated neurofilament epitopes in ballooned neurons of the extraocular muscle nuclei in a case of Werdnig-Hoffmann disease [Elektronische Daten] [S. Kato, A. Hirano] Summary: The extraocular muscle nuclei in one case of Werdnig-Hoffmann disease were examined immunocytochemically using antibodies against phosphorylated neurofilament (pNF) and ubiquitin (UBQ). The oculomotor and trochlear nuclei showed several chromatolytic ballooned neurons. All ballooned neurons contained epitopes of pNF and UBQ. pNF were present mainly in the periphery of the cell in a ring-like shape and were occasionally seen in the center of some cells. On the other hand, the structures stained by the antibody to UBQ were small vesicles or granules and most of them were aggregated in the center of the cell. These distribution patterns of pNF and UBQ may be unique in Werdnig-Hoffmann disease, since similar patterns were reported in other types of neurons of Werdnig-Hoffmann disease but were not seen in two other motor neuron diseases: classical amyotrophic lateral sclerosis, and familial amyotrophic lateral sclerosis with posterior column and spinocerebellar tract involvement. Springer-Verlag, 1990 Werdnig-Hoffmann disease nationallicence Extraocular muscle nuclei nationallicence Chromatolytic neuron nationallicence Phosphory-lated neurofilament nationallicence Ubiquitin nationallicence Kato S. Division of Neuropathology, Department of Pathology, Montefiore Medical Center, 111 East 210th Street, 10467, Bronx, NY, USA aut Hirano A. Division of Neuropathology, Department of Pathology, Montefiore Medical Center, 111 East 210th Street, 10467, Bronx, NY, USA aut Acta Neuropathologica Springer-Verlag 80/3(1990-07-01), 334-337 0001-6322 80:3<334 1990 80 401 https://doi.org/10.1007/BF00294653 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/BF00294653 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Kato S. Division of Neuropathology, Department of Pathology, Montefiore Medical Center, 111 East 210th Street, 10467, Bronx, NY, USA aut NATIONALLICENCE 700 1- Hirano A. Division of Neuropathology, Department of Pathology, Montefiore Medical Center, 111 East 210th Street, 10467, Bronx, NY, USA aut NATIONALLICENCE 773 0- Acta Neuropathologica Springer-Verlag 80/3(1990-07-01), 334-337 0001-6322 80:3<334 1990 80 401 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer