Characterization of the lncRNA transcriptome in mESC-derived motor neurons: Implications for FUS-ALS

Biscarini, Silvia; Capauto, Davide; Peruzzi, Giovanna; Lü, Lei; Colantoni, Alessio; Santini, Tiziana; Shneider, Neil A.; Caffarelli, Elisa; Laneve, Pietro; Bozzoni, Irene

doi:10.1016/j.scr.2018.01.037

Cited by 28 publications

(34 citation statements)

References 41 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…However, to date, only one study has established a link between the roles of lncRNAs in MN development and Hox regulation. Using an embryonic stem cell differentiation system, a battery of MN hallmark lncRNAs have been identified [14,156]. Among these MN-hallmark lncRNAs, knockdown of Meg3 leads to the dysregulation of Hox genes whereby caudal Hox gene expression (Hox9~Hox13) is increased but rostral Hox gene expression (Hox1~Hox8) declines in cervical MNs.…”

Section: Lncrnas In the Regulation Of Postmitotic Neuronsmentioning

confidence: 99%

“…By means of an ESC~MN system, several lncRNAs have been shown to be dysregulated in loss-of-function FUS MNs. Compared to FUS +/+ MNs, Lhx1os upregulation and lncMN-1 (2610316D01Rik) and lncMN-2 (5330434G04Rik) downregulation were observed in FUS P517L/P517L and FUS -/-MNs, suggesting that loss of FUS function affects some lncRNAs conserved among mouse and human [14]. A series of lncRNAs that have not been directly implicated in ALS-associated genetic mutations have been identified to participate in ALS contexts.…”

Section: Other Lncrnas Implicated In Alsmentioning

confidence: 99%

See 1 more Smart Citation

Functional Roles of Long Non-coding RNAs in Motor Neuron Development and Disease

Chen

2020

J Biomed Sci

View full text Add to dashboard Cite

Long non-coding RNAs (lncRNAs) have gained increasing attention as they exhibit highly tissue-and cell-type specific expression patterns. LncRNAs are highly expressed in the central nervous system and their roles in the brain have been studied intensively in recent years, but their roles in the spinal motor neurons (MNs) are largely unexplored. Spinal MN development is controlled by precise expression of a gene regulatory network mediated spatiotemporally by transcription factors, representing an elegant paradigm for deciphering the roles of lncRNAs during development. Moreover, many MN-related neurodegenerative diseases, such as amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA), are associated with RNA metabolism, yet the link between MN-related diseases and lncRNAs remains obscure. In this review, we summarize lncRNAs known to be involved in MN development and disease, and discuss their potential future therapeutic applications.

show abstract

Section: Lncrnas In the Regulation Of Postmitotic Neuronsmentioning

confidence: 99%

Section: Other Lncrnas Implicated In Alsmentioning

confidence: 99%

Functional Roles of Long Non-coding RNAs in Motor Neuron Development and Disease

Chen

2020

J Biomed Sci

View full text Add to dashboard Cite

show abstract

“…4A). Among the top-5 DE lncRNAs, we found Pantr1 and Lhx1os , known to be involved in neuronal development (Biscarini et al, 2018; Carelli et al, 2019).…”

Section: Resultsmentioning

confidence: 99%

One-shot analysis of translated mammalian lncRNAs with AHARIBO

Minati¹,

Firrito²,

Piano³

et al. 2020

Preprint

View full text Add to dashboard Cite

SUMMARYA vast portion of the mammalian genome is transcribed as long non-coding RNAs (lncRNAs) acting in the cytoplasm with largely unknown functions. Surprisingly, lncRNAs have been shown to interact with ribosomes, encode uncharacterized proteins, or act as ribosome sponges. These functions still remain mostly undetected and understudied owing to the lack of efficient tools for genome-wide simultaneous identification of ribosome-associated lncRNAs and peptide-producing lncRNAs.Here we present AHARIBO, a method for the detection of lncRNAs either untranslated, but associated with ribosomes, or encoding small peptides. Using AHARIBO in mouse embryonic stem cells during neuronal differentiation, we isolated ribosome-protected RNA fragments, translated RNAs and corresponding de novo synthesized polypeptides. Besides identifying mRNAs under active translation and associated ribosomes, we found and distinguished lncRNAs acting as ribosome sponges or encoding micropeptides, laying the ground for a better functional understanding of hundreds lncRNAs.

show abstract

“…Furthermore, dysregulated ncRNAs (Lhx1as, lncMN-1, and lncMN2) have been identified in FUS-P517L ALS mouse models, but the consequences are unknown [138]. CircRNAs are also known to be dysregulated in FUS-mutated cells and motor neurons derived from ALS-iPSCs, but the functional roles of those circRNAs are still unclear as well [139].…”

Section: Ncrnas In Other Mechanisms Of Ad Pathogenesismentioning

confidence: 99%

Functional roles and networks of non-coding RNAs in the pathogenesis of neurodegenerative diseases

2020

View full text Add to dashboard Cite

Recent transcriptome analyses have revealed that noncoding RNAs (ncRNAs) are broadly expressed in mammalian cells and abundant in the CNS, with tissue and cell type-specific expression patterns. Moreover, ncRNAs have been found to intricately and dynamically regulate various signaling pathways in neurodegeneration. As such, some antisense transcripts and microRNAs are known to directly affect neurodegeneration in disease contexts. The functions of ncRNAs in pathogenesis are unique for each disorder, as are the pertinent networks of ncRNA/miRNA/ mRNA that mediate these functions. Thus, further understanding of ncRNA biogenesis and effects might aid the discovery of diagnostic biomarkers or development of effective therapeutics for neurodegenerative disorders. Here, we review the ncRNAs that have so far been identified in major neurodegenerative disease etiology and the mechanisms that link ncRNAs with disease-specific phenotypes, such as HTT aggregation in HD, α-synuclein in PD, and Aβ plaques and hyperphosphorylated Tau in AD. We also summarize the known lncRNA/miRNA/mRNA networks that participate in neurodegenerative diseases, and we discuss ncRNA-related treatments shown to delay disease onset and prolong lifespan in rodent models.

show abstract

Characterization of the lncRNA transcriptome in mESC-derived motor neurons: Implications for FUS-ALS

Cited by 28 publications

References 41 publications

Functional Roles of Long Non-coding RNAs in Motor Neuron Development and Disease

Functional Roles of Long Non-coding RNAs in Motor Neuron Development and Disease

One-shot analysis of translated mammalian lncRNAs with AHARIBO

Functional roles and networks of non-coding RNAs in the pathogenesis of neurodegenerative diseases

Contact Info

Product

Resources

About