Pregled bibliografske jedinice broj: 666078
Species-dependent posttranscriptional regulation of NOS1 by FMRP in the developing cerebral cortex
Species-dependent posttranscriptional regulation of NOS1 by FMRP in the developing cerebral cortex // Cell, 149 (2012), 4; 899-911 doi:10.1016/j.cell.2012.02.060 (međunarodna recenzija, članak, znanstveni)
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Naslov
Species-dependent posttranscriptional regulation of NOS1 by FMRP in the developing cerebral cortex
Autori
Kwan, Kenneth Y. ; Lam, Mandy M.S. ; Johnson, Matthew B. ; Dube, Umber ; Shim, Sungbo ; Rašin, Mladen-Roko ; Sousa, André M.M. ; Fertuzinhos, Sofia ; Chen, Jie-Guang ; Arellano, Jon I. ; Chan, Daniel W. ; Pletikos, Mihovil ; Vasung, Lana ; Rowitch, David H. ; Huang, Eric J. ; Schwartz, Michael L. ; Willemsen, Rob ; Oostra, Ben A. ; Rakić, Paško ; Heffer, Marija ; Kostović, Ivica ; Judaš, Miloš ; Šestan, Nenad
Izvornik
Cell (0092-8674) 149
(2012), 4;
899-911
Vrsta, podvrsta i kategorija rada
Radovi u časopisima, članak, znanstveni
Ključne riječi
cerebral cortex ; development ; NOS1 ; FMRP
Sažetak
Fragile X syndrome (FXS), the leading monogenic cause of intellectual disability and autism, results from loss of function of the RNA-binding protein FMRP. Here, we show that FMRP regulates translation of neuronal nitric oxide synthase 1 (NOS1) in the developing human neocortex. Whereas NOS1 mRNA is widely expressed, NOS1 protein is transiently coexpressed with FMRP during early synaptogenesis in layer- and region-specific pyramidal neurons. These include midfetal layer 5 subcortically projecting neurons arranged into alternating columns in the prospective Broca's area and orofacial motor cortex. Human NOS1 translation is activated by FMRP via interactions with coding region binding motifs absent from mouse Nos1 mRNA, which is expressed in mouse pyramidal neurons, but not efficiently translated. Correspondingly, neocortical NOS1 protein levels are severely reduced in developing human FXS cases, but not FMRP-deficient mice. Thus, alterations in FMRP posttranscriptional regulation of NOS1 in developing neocortical circuits may contribute to cognitive dysfunction in FXS.
Izvorni jezik
Engleski
Znanstvena područja
Temeljne medicinske znanosti
POVEZANOST RADA
Projekti:
108-1081870-1878 - Uloga prolaznih fetalnih neurona u razvojnim poremećajima moždane kore (Judaš, Miloš, MZOS ) ( CroRIS)
108-1081870-1876 - Razvitak kortikalnih putova u čovjeka (Kostović, Ivica, MZOS ) ( CroRIS)
Ustanove:
Medicinski fakultet, Zagreb,
Sveučilište u Osijeku - Odjel za biologiju
Profili:
Lana Vasung
(autor)
Ivica Kostović
(autor)
Marija Heffer
(autor)
Mihovil Pletikos
(autor)
Miloš Judaš
(autor)
Citiraj ovu publikaciju:
Časopis indeksira:
- Current Contents Connect (CCC)
- Web of Science Core Collection (WoSCC)
- Science Citation Index Expanded (SCI-EXP)
- SCI-EXP, SSCI i/ili A&HCI
- Scopus
- MEDLINE