UBIQUITIN AND PHOSPHORYLATED NEUROFILAMENT EPITOPES IN BALLOONED NEURONS OF THE EXTRAOCULAR-MUSCLE NUCLEI IN A CASE OF WERDNIG-HOFFMANN DISEASE

被引:31
作者
KATO, S [1 ]
HIRANO, A [1 ]
机构
[1] MONTEFIORE MED CTR, DEPT PATHOL, DIV NEUROPATHOL, 111 E 210TH ST, BRONX, NY 10467 USA
关键词
Chromatolytic neuron; Extraocular muscle nuclei; Phosphory-lated neurofilament; Ubiquitin; Werdnig-Hoffmann disease;
D O I
10.1007/BF00294653
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
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. © 1990 Springer-Verlag.
引用
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页码:334 / 337
页数:4
相关论文
共 24 条
[1]   PATHOLOGICAL INVOLVEMENT OF PRIMARY SENSORY NEURONS IN WERDNIG-HOFFMANN DISEASE [J].
CARPENTER, S ;
KARPATI, G ;
ROTHMAN, S ;
WATTERS, G ;
ANDERMANN, F .
ACTA NEUROPATHOLOGICA, 1978, 42 (02) :91-97
[2]   ULTRASTRUCTURE OF CHROMATOLYTIC MOTONEURONS AND ANTERIOR SPINAL ROOTS IN A CASE OF WERDNIG-HOFFMANN DISEASE [J].
CHOU, SM ;
FAKADEJ, AV .
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 1971, 30 (03) :368-&
[3]   BALLOONED NEURONS IN SELECT NEURODEGENERATIVE DISEASES CONTAIN PHOSPHORYLATED NEUROFILAMENT EPITOPES [J].
DICKSON, DW ;
YEN, SH ;
SUZUKI, KI ;
DAVIES, P ;
GARCIA, JH ;
HIRANO, A .
ACTA NEUROPATHOLOGICA, 1986, 71 (3-4) :216-223
[4]   REACTION OF LEWY BODIES WITH ANTIBODIES TO PHOSPHORYLATED AND NONPHOSPHORYLATED NEUROFILAMENTS [J].
FORNO, LS ;
STERNBERGER, LA ;
STERNBERGER, NH ;
STREFLING, AM ;
SWANSON, K ;
ENG, LF .
NEUROSCIENCE LETTERS, 1986, 64 (03) :253-258
[5]   LEWY BODIES OF PARKINSONS-DISEASE CONTAIN NEUROFILAMENT ANTIGENS [J].
GOLDMAN, JE ;
YEN, SH ;
CHIU, FC ;
PERESS, NS .
SCIENCE, 1983, 221 (4615) :1082-1084
[6]   ACCUMULATION OF PHOSPHORYLATED NEUROFILAMENTS IN THE ANTERIOR HORN MOTOR NEURONS OF ALS PATIENTS [J].
GREENE, C ;
MUNOZGARCIA, D ;
PERL, DP ;
PENDLEBURY, W .
JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY, 1986, 45 (03) :332-332
[7]   UBIQUITIN - ROLES IN PROTEIN MODIFICATION AND BREAKDOWN [J].
HERSHKO, A .
CELL, 1983, 34 (01) :11-12
[8]  
Hirano A, 1982, Adv Neurol, V36, P75
[9]  
HIRANO A, 1980, NEUROL MED TOKYO, V13, P148
[10]  
IWATA M, 1978, NEUROL MED CHIR TOKY, V8, P40