prikaz prve stranice dokumenta Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy
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Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy
Acta neuropathologica, 115 (2008), 3; 313-326. https://doi.org/10.1007/s00401-007-0327-1
Šimić, Goran; Mladinov, Mihovil; Šešo Šimić, Đurđica; Jovanov Milošević, Nataša; Islam, Atiqul; Pajtak, Alen; Barišić, Nina; Sertić, Jadranka; Lucassen, Paul J.; Hof, Patrick R.; Krušlin, Božo

Citirajte ovaj rad

Šimić, G., Mladinov, M., Šešo Šimić, Đ., Jovanov Milošević, N., Islam, A., Pajtak, A. ... Krušlin, B. (2008). Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy. Acta neuropathologica, 115. (3), 313-326. doi: 10.1007/s00401-007-0327-1

Šimić, Goran, et al. "Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy." Acta neuropathologica, vol. 115, br. 3, 2008, str. 313-326. https://doi.org/10.1007/s00401-007-0327-1

Šimić, Goran, Mihovil Mladinov, Đurđica Šešo Šimić, Nataša Jovanov Milošević, Atiqul Islam, Alen Pajtak, Nina Barišić, et al. "Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy." Acta neuropathologica 115, br. 3 (2008): 313-326. https://doi.org/10.1007/s00401-007-0327-1

Šimić, G., et al. (2008) 'Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy', Acta neuropathologica, 115(3), str. 313-326. doi: 10.1007/s00401-007-0327-1

Šimić G, Mladinov M, Šešo Šimić Đ, Jovanov Milošević N, Islam A, Pajtak A, i sur.. Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy. Acta neuropathologica [Internet]. 03.2008. [pristupljeno 12.04.2024.];115(3):313-326. doi: 10.1007/s00401-007-0327-1

G. Šimić, et al., "Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy", Acta neuropathologica, vol. 115, br. 3, str. 313-326, Ožujak 2008. [Online]. Dostupno na: https://urn.nsk.hr/urn:nbn:hr:105:624836. [Citirano: 12.04.2024.]

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Podaci o radu
Naslov (engleski)Abnormal motoneuron migration, differentiation, and axon outgrowth in spinal muscular atrophy
AutorGoran Šimić
AutorMihovil Mladinov
AutorĐurđica Šešo Šimić
AutorNataša Jovanov Milošević
AutorAtiqul Islam
AutorAlen Pajtak
AutorNina Barišić
AutorJadranka Sertić
AutorPaul J. Lucassen
AutorPatrick R. Hof
AutorBožo Krušlin
Autorova ustanovaSveučilište u Zagrebu
Medicinski fakultet
Znanstveno / umjetničko
područje, polje i grana		BIOMEDICINA I ZDRAVSTVO
Kliničke medicinske znanosti
Sažetak (engleski)
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.
Ključne riječi (engleski)
Jezikengleski
Vrsta publikacijeZnanstveni rad - Izvorni znanstveni rad
Status objaveObjavljen
Vrsta recenzijeRecenziran
Verzija publikacijeZavršna verzija rukopisa prihvaćena za objavljivanje (postprint)
Naslov časopisaActa neuropathologica
Brojčani podacivol. 115, br. 3, str. 313-326
p-ISSN0001-6322
DOIhttps://doi.org/10.1007/s00401-007-0327-1
URN:NBNurn:nbn:hr:105:624836
Datum objave publikacije2008-03
URL dokumentahttps://doi.org/10.1007/s00401-007-0327-1
Vrsta resursaTekst
Prava pristupaOtvoreni pristup
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Datum i vrijeme pohrane2024-03-28 10:04:40