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Pregled bibliografske jedinice broj: 323041


Autori: Šimić, Goran; Mladinov, Mihovil; Šešo-Šimić, Đurđica; Jovanov-Milošević, Nataša; Islam, Atiqul; Pajtak, Alen; Barišić, Nina; Sertić, Jadranka; Lucassen J. Paul; Hof R. Patrick; Krušlin, Božo
Naslov: Abnormal motoneuron migration, differentiation and axon outgrowth in spinal muscular atrophy
Izvornik: Acta neuropathologica (0001-6322) 115 (2008), 3; 313-326
Vrsta rada: članak
Ključne riječi: motoneurons; migration; pathogenesis; spinal muscular atrophy; SMN1 gene
The role of heterotopic (migratory) motoneurons (HMN) in the pathogenesis of spinal muscular atrophy(SMA) is still controversial. We examined the occurrence and amount of HMN in spinal cord tissue from eight children with SMA (six with SMA-I and two with SMA-II). All affected subjects were carrying a homozygous deletion of exon 7 in the SMN1 gene. Unlike controls, virtually free from HMN, all SMA subjects showed a significant number of HMN at all levels of the spinal cord. Heterotopic neurons were hyperchromatic, located mostly in the ventral white matter and had no axon or dendrites. More than half of the HMN were very undifferentiated, as judged from their lack of immunoreactivity for NeuN and MAP2 proteins. Small numbers of more differentiated heterotopic neurons were also found in the dorsal and lateral white matter region. As confirmed by ultrastructural analysis, in situ end labeling (ISEL) and CD68 immunoreactivity, HMN in the ventral outflow were found to have no synapses, to activate microglial cells, and to eventually die by necrosis. An unbiased quantitative analysis showed a significant negative correlation between age of SMA subjects (a reflection of the clinical severity) and the number of HMN. Subjects who died at older ages had increased number of GFAP-positive astrocytes. Complementing our previous report on motoneuron apoptosis within the ventral horns in SMA, we now propose that abnormal migration, differentiation, and lack of axonal outgrowth may induce motoneuron apoptosis predominantly during early stages, whereas a slower necrosis-like cell death of displaced motoneurons which ‘ escaped’ apoptosis characterizes later stages of SMA.
Projekt / tema: 108-1081870-1884, 108-1081870-1942, 108-1081870-1886
Izvorni jezik: ENG
Rad je indeksiran u
bazama podataka:
Current Contents Connect (CCC)
Science Citation Index Expanded (SCI-EXP) (sastavni dio Web of Science Core Collectiona)
Kategorija: Znanstveni
Znanstvena područja:
Temeljne medicinske znanosti,Kliničke medicinske znanosti
Puni text rada: 323041.fulltext.pdf (tekst priložen 22. Vel. 2008. u 13:24 sati)
URL Internet adrese:
Broj citata:
DOI: 10.1007/s00401-007-0327-1
URL cjelovitog teksta:
Google Scholar: Abnormal motoneuron migration, differentiation and axon outgrowth in spinal muscular atrophy
Upisao u CROSBI: Goran Šimić (, 22. Vel. 2008. u 13:12 sati

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