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Changes in expression of the long non-coding RNA FMR4 associate with altered gene expression during differentiation of human neural precursor cells.

Publication ,  Journal Article
Peschansky, VJ; Pastori, C; Zeier, Z; Motti, D; Wentzel, K; Velmeshev, D; Magistri, M; Bixby, JL; Lemmon, VP; Silva, JP; Wahlestedt, C
Published in: Front Genet
2015

CGG repeat expansions in the Fragile X mental retardation 1 (FMR1) gene are responsible for a family of associated disorders characterized by either intellectual disability and autism Fragile X Syndrome (FXS), or adult-onset neurodegeneration Fragile X-associated Tremor/Ataxia Syndrome. However, the FMR1 locus is complex and encodes several long non-coding RNAs, whose expression is altered by repeat expansion mutations. The role of these lncRNAs is thus far unknown; therefore we investigated the functionality of FMR4, which we previously identified. "Full"-length expansions of the FMR1 triplet repeat cause silencing of both FMR1 and FMR4, thus we are interested in potential loss-of-function that may add to phenotypic manifestation of FXS. Since the two transcripts do not exhibit cis-regulation of one another, we examined the potential for FMR4 to regulate target genes at distal genomic loci using gene expression microarrays. We identified FMR4-responsive genes, including the methyl-CpG-binding domain protein 4 (MBD4). Furthermore, we found that in differentiating human neural precursor cells, FMR4 expression is developmentally regulated in opposition to expression of both FMR1 (which is expected to share a bidirectional promoter with FMR4) and MBD4. We therefore propose that FMR4's function is as a gene-regulatory lncRNA and that this transcript may function in normal development. Closer examination of FMR4 increases our understanding of the role of regulatory lncRNA and the consequences of FMR1 repeat expansions.

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Published In

Front Genet

DOI

ISSN

1664-8021

Publication Date

2015

Volume

6

Start / End Page

263

Location

Switzerland

Related Subject Headings

  • 3105 Genetics
  • 1801 Law
  • 1103 Clinical Sciences
  • 0604 Genetics
 

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APA
Chicago
ICMJE
MLA
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Peschansky, V. J., Pastori, C., Zeier, Z., Motti, D., Wentzel, K., Velmeshev, D., … Wahlestedt, C. (2015). Changes in expression of the long non-coding RNA FMR4 associate with altered gene expression during differentiation of human neural precursor cells. Front Genet, 6, 263. https://doi.org/10.3389/fgene.2015.00263
Peschansky, Veronica J., Chiara Pastori, Zane Zeier, Dario Motti, Katya Wentzel, Dmitry Velmeshev, Marco Magistri, et al. “Changes in expression of the long non-coding RNA FMR4 associate with altered gene expression during differentiation of human neural precursor cells.Front Genet 6 (2015): 263. https://doi.org/10.3389/fgene.2015.00263.
Peschansky VJ, Pastori C, Zeier Z, Motti D, Wentzel K, Velmeshev D, et al. Changes in expression of the long non-coding RNA FMR4 associate with altered gene expression during differentiation of human neural precursor cells. Front Genet. 2015;6:263.
Peschansky, Veronica J., et al. “Changes in expression of the long non-coding RNA FMR4 associate with altered gene expression during differentiation of human neural precursor cells.Front Genet, vol. 6, 2015, p. 263. Pubmed, doi:10.3389/fgene.2015.00263.
Peschansky VJ, Pastori C, Zeier Z, Motti D, Wentzel K, Velmeshev D, Magistri M, Bixby JL, Lemmon VP, Silva JP, Wahlestedt C. Changes in expression of the long non-coding RNA FMR4 associate with altered gene expression during differentiation of human neural precursor cells. Front Genet. 2015;6:263.

Published In

Front Genet

DOI

ISSN

1664-8021

Publication Date

2015

Volume

6

Start / End Page

263

Location

Switzerland

Related Subject Headings

  • 3105 Genetics
  • 1801 Law
  • 1103 Clinical Sciences
  • 0604 Genetics