Naslov
Are heterotopic motoneurons in spinal muscular atrophy actually adult stem cells?

Autori
Šimić, Goran ; Mladinov, Mihovil ; Diana, Andrea ; Krušlin, Božo

Vrsta, podvrsta i kategorija rada
Sažeci sa skupova, sažetak, znanstveni

Izvornik
FENS Forum Abstracts / Morris, Richard - Ženeva : Federation of European Neuroscience Societies (FENS), 2008, 239-239

ISBN
92-990014-3-X

Skup
6th FENS Forum of European Neuroscience

Mjesto i datum
Ženeva, Švicarska, 12.07.2008. - 16.07.2008

Vrsta sudjelovanja
Poster

Vrsta recenzije
Međunarodna recenzija

Ključne riječi
migration; motoneurons; neural stem cells; SMN1 gene; SMN protein; spinal muscular atrophy

Sažetak
The origin and role of heterotopic (migratory) motoneurons (HMN) in the pathogenesis of spinal muscular atrophy (SMA) is still a matter of debate. In our recent paper (Simic et al., Acta Neuropathol. 2008) we have shown that, unlike controls, SMA patients with confirmed homozygous deletion of exon 7 in the SMN1 gene display a significant number of HMN at all levels of spinal cord. A quantitative analysis showed a negative correlation between the number of HMN and age of SMA subjects (a reflection of the clinical severity). HMN are located in the ventral white matter and anterior roots, have no axon or dendrites and look hyperchromatic in Nissl-stained sections. Since most of them show immunonegativity for markers of mature, differentiated neurons (e.g. NeuN and MAP2 proteins), this raises the possibility that some of them may represent resident adult stem cells. We hope that the analysis of nestin (which is expressed predominantly in neural stem cells and not in mature cells of the central nervous system) and developmental markers of motor neurons (such as islet, lim and HB9) in postmortem spinal cord tissue of genetically confirmed SMA patients will be sufficient to give an adequate answer to this hypothesis.

Izvorni jezik
Engleski

Znanstvena područja
Biologija, Temeljne medicinske znanosti, Kliničke medicinske znanosti

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