Correction: Corrigendum: Angelman syndrome-derived neurons display late onset of paternal UBE3A silencing

Staňurová, Jana; Neureiter, Anika; Hiber, Michaela; Kessler, Hannah de Oliveira; Stolp, Kristin; Goetzke, Roman; Klein, Diana; Bànkfalvi, Àgnes; Klump, Hannes; Steenpaß, Laura

doi:10.1038/srep46952

Cited by 2 publications

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“…On the paternal allele of imprinted human PWS/AS locus, the unmethylated PWS-ICR is the region upstream to a protein-coding gene SNRPN and a lncRNA SNHG14 (small nucleolar RNA host gene 14) ( Sutcliffe et al, 1994 ; Buiting et al, 1995 ; Rougeulle et al, 1997 ; Runte et al, 2001 ; Vitali et al, 2010 ; Chamberlain, 2013 ; Stanurova et al, 2018 ; Figure 4A ). The neuron-specific non-coding transcript SNHG14 is processed to give rise to a series of non-coding RNA products, such as repeated C/D box small nucleolar RNAs (snoRNAs) and lncRNAs including 116HG , 115HG , and the antisense transcript to UBE3A ( Mendiola and LaSalle, 2021 ; Figure 4A ).…”

Section: The Role Of Imprinted Long Non-coding Rnas In Human Imprinting Disorders and Cancermentioning

confidence: 99%

“…The neuron-specific non-coding transcript SNHG14 is processed to give rise to a series of non-coding RNA products, such as repeated C/D box small nucleolar RNAs (snoRNAs) and lncRNAs including 116HG , 115HG , and the antisense transcript to UBE3A ( Mendiola and LaSalle, 2021 ; Figure 4A ). The most studied RNA product from the host transcript SNHG14 is SNORD116 snoRNA, embedded within intronic regions of SNORD116 gene locus ( Cavaillé et al, 2000 ; de los Santos et al, 2000 ; Stanurova et al, 2018 ; Mendiola and LaSalle, 2021 ). SNORD116 snoRNA present in ribonucleoprotein complexes (snoRNPs) and may participate in splicing, ribosomal RNA maturation, RNA modifications, and regulation of prohormone processing-related gene expression ( Bazeley et al, 2008 ; Burnett et al, 2017 ).…”

Section: The Role Of Imprinted Long Non-coding Rnas In Human Imprinting Disorders and Cancermentioning

confidence: 99%

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The Role of Long Non-coding RNAs in Human Imprinting Disorders: Prospective Therapeutic Targets

Wang

Jianjian

Yang

et al. 2021

Front. Cell Dev. Biol.

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Genomic imprinting is a term used for an intergenerational epigenetic inheritance and involves a subset of genes expressed in a parent-of-origin-dependent way. Imprinted genes are expressed preferentially from either the paternally or maternally inherited allele. Long non-coding RNAs play essential roles in regulating this allele-specific expression. In several well-studied imprinting clusters, long non-coding RNAs have been found to be essential in regulating temporal- and spatial-specific establishment and maintenance of imprinting patterns. Furthermore, recent insights into the epigenetic pathological mechanisms underlying human genomic imprinting disorders suggest that allele-specific expressed imprinted long non-coding RNAs serve as an upstream regulator of the expression of other protein-coding or non-coding imprinted genes in the same cluster. Aberrantly expressed long non-coding RNAs result in bi-allelic expression or silencing of neighboring imprinted genes. Here, we review the emerging roles of long non-coding RNAs in regulating the expression of imprinted genes, especially in human imprinting disorders, and discuss three strategies targeting the central long non-coding RNA UBE3A-ATS for the purpose of developing therapies for the imprinting disorders Prader–Willi syndrome and Angelman syndrome. In summary, a better understanding of long non-coding RNA-related mechanisms is key to the development of potential therapeutic targets for human imprinting disorders.

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Section: The Role Of Imprinted Long Non-coding Rnas In Human Imprinting Disorders and Cancermentioning

confidence: 99%

Section: The Role Of Imprinted Long Non-coding Rnas In Human Imprinting Disorders and Cancermentioning

confidence: 99%

The Role of Long Non-coding RNAs in Human Imprinting Disorders: Prospective Therapeutic Targets

Wang

Jianjian

Yang

et al. 2021

Front. Cell Dev. Biol.

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“…In humans, SNORD115 , but not SNORD116 or UBE3A-ATS , is exclusively expressed in neurons, while Snord116 , Snord115 , and Ube3a-ats are all neuron-specific transcripts in mouse. SNORD115 and SNORD116 encompass clusters of repeated subunits of sequences encoding a C/D box snoRNAs embedded within intronic regions of the noncoding exons encoding the snoRNA host transcript SNHG14 ( Cavaillé et al, 2000 ; de los Santos et al, 2000 ; Bortolin-Cavaillé and Cavaillé, 2012 ; Stanurova et al, 2018 ). C/D box snoRNAs have known functions in regulating 2-O methylation rRNA modifications by recruiting ribonucleoprotein complexes including fibrillarin, which catalyzes methylation ( Dupuis-Sandoval et al, 2015 ; Bratkovič et al, 2020 ).…”

Section: Introductionmentioning

confidence: 99%

Epigenetics in Prader-Willi Syndrome

Mendiola

LaSalle

2021

Front. Genet.

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Prader-Willi Syndrome (PWS) is a rare neurodevelopmental disorder that affects approximately 1 in 20,000 individuals worldwide. Symptom progression in PWS is classically characterized by two nutritional stages. Stage 1 is hypotonia characterized by poor muscle tone that leads to poor feeding behavior causing failure to thrive in early neonatal life. Stage 2 is followed by the development of extreme hyperphagia, also known as insatiable eating and fixation on food that often leads to obesity in early childhood. Other major features of PWS include obsessive-compulsive and hoarding behaviors, intellectual disability, and sleep abnormalities. PWS is genetic disorder mapping to imprinted 15q11.2-q13.3 locus, specifically at the paternally expressed SNORD116 locus of small nucleolar RNAs and noncoding host gene transcripts. SNORD116 is processed into several noncoding components and is hypothesized to orchestrate diurnal changes in metabolism through epigenetics, according to functional studies. Here, we review the current status of epigenetic mechanisms in PWS, with an emphasis on an emerging role for SNORD116 in circadian and sleep phenotypes. We also summarize current ongoing therapeutic strategies, as well as potential implications for more common human metabolic and psychiatric disorders.

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Correction: Corrigendum: Angelman syndrome-derived neurons display late onset of paternal UBE3A silencing

Cited by 2 publications

References 0 publications

The Role of Long Non-coding RNAs in Human Imprinting Disorders: Prospective Therapeutic Targets

The Role of Long Non-coding RNAs in Human Imprinting Disorders: Prospective Therapeutic Targets

Epigenetics in Prader-Willi Syndrome

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