Chromatolytic neurons in Werdnig-Hoffmann disease contain phosphorylated neurofilaments
- PMID: 3149123
- DOI: 10.1007/BF00688247
Chromatolytic neurons in Werdnig-Hoffmann disease contain phosphorylated neurofilaments
Abstract
Two cases of Werdnig-Hoffman disease are described. Chromatolytic neurons were found within the ventral horns of the spinal cord as well as in dorsal root ganglia, Clarke's column, cranial nerve nuclei and thalamus. Immunohistochemical staining demonstrated phosphorylated neurofilament antigen within the cytoplasm of the chromatolytic neurons. This finding suggests that a defect in slow axonal transport or in the regulation of neurofilament phosphorylation may play a role in the pathogenesis of this disorder.
Similar articles
-
Ubiquitin and phosphorylated neurofilament epitopes in ballooned neurons of the extraocular muscle nuclei in a case of Werdnig-Hoffmann disease.Acta Neuropathol. 1990;80(3):334-7. doi: 10.1007/BF00294653. Acta Neuropathol. 1990. PMID: 1698008
-
Aberrant glycosylation/phosphorylation in chromatolytic motoneurons of Werdnig-Hoffmann disease.J Neurol Sci. 1997 Nov 25;152(2):198-209. doi: 10.1016/s0022-510x(97)00161-5. J Neurol Sci. 1997. PMID: 9415542
-
A study of infantile motor neuron disease with neurofilament and ubiquitin immunocytochemistry.Neuropediatrics. 1989 May;20(2):107-11. doi: 10.1055/s-2008-1071275. Neuropediatrics. 1989. PMID: 2544822
-
Controversy over Werdnig-Hoffmann disease and multiple system atrophy.Curr Opin Neurol. 1993 Dec;6(6):861-4. doi: 10.1097/00019052-199312000-00006. Curr Opin Neurol. 1993. PMID: 8293160 Review. No abstract available.
-
[Two siblings of familial amyotrophic lateral sclerosis with multisystemic degeneration characterized by mild involvement of the middle root zone of the posterior column, Clarke's nuclei and spinocerebellar tract].Rinsho Shinkeigaku. 1995 Jun;35(6):589-99. Rinsho Shinkeigaku. 1995. PMID: 8521632 Review. Japanese.
Cited by
-
Ubiquitin and phosphorylated neurofilament epitopes in ballooned neurons of the extraocular muscle nuclei in a case of Werdnig-Hoffmann disease.Acta Neuropathol. 1990;80(3):334-7. doi: 10.1007/BF00294653. Acta Neuropathol. 1990. PMID: 1698008
-
A reassessment of spinal cord pathology in severe infantile spinal muscular atrophy: Reassessment of spinal cord pathology.Neuropathol Appl Neurobiol. 2024 Oct;50(5):e13013. doi: 10.1111/nan.13013. Neuropathol Appl Neurobiol. 2024. PMID: 39449271 Free PMC article.
-
Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells.Acta Neuropathol. 1990;79(4):402-8. doi: 10.1007/BF00308716. Acta Neuropathol. 1990. PMID: 2111074
-
Immunocytochemical and ultrastructural studies of Werdnig-Hoffmann disease.Acta Neuropathol. 1991;81(4):408-17. doi: 10.1007/BF00293462. Acta Neuropathol. 1991. PMID: 1851364
-
Failure of lower motor neuron radial outgrowth precedes retrograde degeneration in a feline model of spinal muscular atrophy.J Comp Neurol. 2012 Jun 1;520(8):1737-50. doi: 10.1002/cne.23010. J Comp Neurol. 2012. PMID: 22120001 Free PMC article.
References
Publication types
MeSH terms
Substances
LinkOut - more resources
Medical