{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T20:13:46Z","timestamp":1769631226030,"version":"3.49.0"},"reference-count":88,"publisher":"MDPI AG","issue":"15","license":[{"start":{"date-parts":[[2022,8,5]],"date-time":"2022-08-05T00:00:00Z","timestamp":1659657600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft (DFG)","doi-asserted-by":"publisher","award":["RO5622\/1-1"],"award-info":[{"award-number":["RO5622\/1-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100001659","name":"Deutsche Forschungsgemeinschaft (DFG)","doi-asserted-by":"publisher","award":["SK259\/2-1"],"award-info":[{"award-number":["SK259\/2-1"]}],"id":[{"id":"10.13039\/501100001659","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["IJMS"],"abstract":"<jats:p>Prader\u2013Willi syndrome (PWS) is a complex neurodevelopmental disorder caused by the deletion or inactivation of paternally expressed imprinted genes at the chromosomal region 15q11\u2013q13. The PWS-critical region (PWScr) harbors tandemly repeated non-protein coding IPW-A exons hosting the intronic SNORD116 snoRNA gene array that is predominantly expressed in brain. Paternal deletion of PWScr is associated with key PWS symptoms in humans and growth retardation in mice (PWScr model). Dysregulation of the hypothalamic\u2013pituitary axis (HPA) is thought to be causally involved in the PWS phenotype. Here we performed a comprehensive reverse transcription quantitative PCR (RT-qPCR) analysis across nine different brain regions of wild-type (WT) and PWScr mice to identify stably expressed reference genes. Four methods (Delta Ct, BestKeeper, Normfinder and Genorm) were applied to rank 11 selected reference gene candidates according to their expression stability. The resulting panel consists of the top three most stably expressed genes suitable for gene-expression profiling and comparative transcriptome analysis of WT and\/or PWScr mouse brain regions. Using these reference genes, we revealed significant differences in the expression patterns of Igfbp7, Nlgn3 and three HPA associated genes: Pcsk1,\u00a0Pcsk2 and Nhlh2 across investigated brain regions of wild-type and PWScr mice. Our results raise a reasonable doubt on the involvement of the Snord116 in posttranscriptional regulation of Nlgn3 and Nhlh2 genes. We provide a valuable tool for expression analysis of specific genes across different areas of the mouse brain and for comparative investigation of PWScr mouse models to discover and verify different regulatory pathways affecting this complex disorder.<\/jats:p>","DOI":"10.3390\/ijms23158729","type":"journal-article","created":{"date-parts":[[2022,8,7]],"date-time":"2022-08-07T22:51:46Z","timestamp":1659912706000},"page":"8729","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Reference Genes across Nine Brain Areas of Wild Type and Prader-Willi Syndrome Mice: Assessing Differences in Igfbp7, Pcsk1, Nhlh2 and Nlgn3 Expression"],"prefix":"10.3390","volume":"23","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2793-5987","authenticated-orcid":false,"given":"Delf-Magnus","family":"Kummerfeld","sequence":"first","affiliation":[{"name":"Medical Faculty, Core Facility Transgenic Animal and Genetic Engineering Models (TRAM), University of Muenster, Von-Esmarch-Str. 56, 48149 Muenster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4392-185X","authenticated-orcid":false,"given":"Boris V.","family":"Skryabin","sequence":"additional","affiliation":[{"name":"Medical Faculty, Core Facility Transgenic Animal and Genetic Engineering Models (TRAM), University of Muenster, Von-Esmarch-Str. 56, 48149 Muenster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Juergen","family":"Brosius","sequence":"additional","affiliation":[{"name":"Institute for Systems Genetics, West China Hospital, Sichuan University, Chengdu 610041, China"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0418-5765","authenticated-orcid":false,"given":"Sergey Y.","family":"Vakhrushev","sequence":"additional","affiliation":[{"name":"Copenhagen Center for Glycomics, Departments of Cellular and Molecular Medicine, Faculty of Health Sciences, University of Copenhagen, 2200 Copenhagen, Denmark"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3384-925X","authenticated-orcid":false,"given":"Timofey S.","family":"Rozhdestvensky","sequence":"additional","affiliation":[{"name":"Medical Faculty, Core Facility Transgenic Animal and Genetic Engineering Models (TRAM), University of Muenster, Von-Esmarch-Str. 56, 48149 Muenster, Germany"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1968","published-online":{"date-parts":[[2022,8,5]]},"reference":[{"key":"ref_1","first-page":"10","article-title":"Prader-Willi syndrome","volume":"14","author":"Cassidy","year":"2012","journal-title":"Genet. Med. Off. J. Am. Coll. Med. Genet."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"14311","DOI":"10.1073\/pnas.250426397","article-title":"Identification of brain-specific and imprinted small nucleolar RNA genes exhibiting an unusual genomic organization","volume":"97","author":"Buiting","year":"2000","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"2687","DOI":"10.1093\/hmg\/10.23.2687","article-title":"The IC-SNURF-SNRPN transcript serves as a host for multiple small nucleolar RNA species and as an antisense RNA for UBE3A","volume":"10","author":"Runte","year":"2001","journal-title":"Hum. Mol. Genet."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"719","DOI":"10.1038\/ng.158","article-title":"Prader-Willi phenotype caused by paternal deficiency for the HBII-85 C\/D box small nucleolar RNA cluster","volume":"40","author":"Sahoo","year":"2008","journal-title":"Nat. Genet."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Skryabin, B.V., Gubar, L.V., Seeger, B., Pfeiffer, J., Handel, S., Robeck, T., Karpova, E., Rozhdestvensky, T., and Brosius, J. (2007). Deletion of the MBII-85 snoRNA Gene Cluster in Mice Results in Postnatal Growth Retardation. PLoS Genet., 3.","DOI":"10.1371\/journal.pgen.0030235"},{"key":"ref_6","doi-asserted-by":"crossref","unstructured":"Kummerfeld, D.-M., Raabe, C., Brosius, J., Mo, D., Skryabin, B., and Rozhdestvensky, T. (2021). A Comprehensive Review of Genetically Engineered Mouse Models for Prader-Willi Syndrome Research. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22073613"},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"6445","DOI":"10.1038\/srep06445","article-title":"Differential regulation of non-protein coding RNAs from Prader-Willi Syndrome locus","volume":"4","author":"Galiveti","year":"2014","journal-title":"Sci. Rep."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"207","DOI":"10.2174\/1573396315666190716120925","article-title":"Prader-Willi Syndrome\u2014Clinical Genetics, Diagnosis and Treatment Approaches: An Update","volume":"15","author":"Butler","year":"2019","journal-title":"Curr. Pediatr. Rev."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1159\/000313377","article-title":"Endocrine Disorders in Children with Prader-Willi Syndrome\u2014Data from 142 Children of the French Database","volume":"74","author":"Diene","year":"2010","journal-title":"Horm. Res. Paediatr."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"274","DOI":"10.21037\/tp.2017.09.04","article-title":"Review of Prader-Willi syndrome: The endocrine approach","volume":"6","author":"Heksch","year":"2017","journal-title":"Transl. Pediatr."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"430","DOI":"10.1002\/ajmg.a.31606","article-title":"Whole genome microarray analysis of gene expression in Prader\u2013Willi syndrome","volume":"143A","author":"Bittel","year":"2007","journal-title":"Am. J. Med Genet. Part A"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1172\/JCI88648","article-title":"Deficiency in prohormone convertase PC1 impairs prohormone processing in Prader-Willi syndrome","volume":"127","author":"Burnett","year":"2016","journal-title":"J. Clin. Investig."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"1664","DOI":"10.1038\/s41436-021-01185-y","article-title":"SNORD116 and growth hormone therapy impact IGFBP7 in Prader\u2013Willi syndrome","volume":"23","author":"Eddiry","year":"2021","journal-title":"Genet. Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"4191","DOI":"10.1210\/en.2007-0173","article-title":"Regulation of Prohormone Convertases in Hypothalamic Neurons: Implications for ProThyrotropin-Releasing Hormone and Proopiomelanocortin","volume":"148","author":"Nillni","year":"2007","journal-title":"Endocrinology"},{"key":"ref_15","doi-asserted-by":"crossref","unstructured":"Mbikay, M., and Seidah, N.G. (2011). The Proprotein Convertases, 20 Years Later. Proprotein Convertases, Humana Press. Methods in Molecular Biology.","DOI":"10.1007\/978-1-61779-204-5"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1438","DOI":"10.1210\/me.2008-0010","article-title":"Nescient Helix-Loop-Helix 2 Interacts with Signal Transducer and Activator of Transcription 3 to Regulate Transcription of Prohormone Convertase 1\/3","volume":"22","author":"Fox","year":"2008","journal-title":"Mol. Endocrinol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1016\/S0031-9384(02)00885-5","article-title":"Reduced voluntary activity precedes adult-onset obesity in Nhlh2 knockout mice","volume":"77","author":"Coyle","year":"2002","journal-title":"Physiol. Behav."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1016\/j.peptides.2012.02.024","article-title":"Biosynthesis of proTRH-derived peptides in prohormone convertase 1 and 2 knockout mice","volume":"35","author":"Cyr","year":"2012","journal-title":"Peptides"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1503","DOI":"10.1210\/en.2003-0834","article-title":"Deletion of the Nhlh2 Transcription Factor Decreases the Levels of the Anorexigenic Peptides? Melanocyte-Stimulating Hormone and Thyrotropin-Releasing Hormone and Implicates Prohormone Convertases I and II in Obesity","volume":"145","author":"Jing","year":"2004","journal-title":"Endocrinology"},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1884","DOI":"10.1093\/hmg\/ddl111","article-title":"Obesity, hyperphagia and increased metabolic efficiency in Pc1 mutant mice","volume":"15","author":"Lloyd","year":"2006","journal-title":"Hum. Mol. Genet."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"4600","DOI":"10.1210\/jc.2009-1831","article-title":"Elevated Insulin-Like Growth Factor-I Values in Children with Prader-Willi Syndrome Compared with Growth Hormone (GH) Deficiency Children over Two Years of GH Treatment","volume":"95","author":"Feigerlova","year":"2010","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"168","DOI":"10.1038\/nature05453","article-title":"Genome-wide atlas of gene expression in the adult mouse brain","volume":"445","author":"Lein","year":"2007","journal-title":"Nature"},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"402","DOI":"10.1006\/meth.2001.1262","article-title":"Analysis of relative gene expression data using real-time quantitative PCR and the 2\u2212\u0394\u0394CT Method","volume":"25","author":"Livak","year":"2001","journal-title":"Methods"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"e45","DOI":"10.1093\/nar\/29.9.e45","article-title":"A new mathematical model for relative quantification in real-time RT-PCR","volume":"29","author":"Pfaffl","year":"2001","journal-title":"Nucleic Acids Res."},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1038\/nprot.2008.73","article-title":"Analyzing real-time PCR data by the comparative CT method","volume":"3","author":"Schmittgen","year":"2008","journal-title":"Nat. Protoc."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"325","DOI":"10.2478\/jomb-2014-0001","article-title":"Gene Expression Studies: How to Obtain Accurate and Reliable Data by Quantitative Real-Time RT PCR","volume":"32","author":"Nestorov","year":"2013","journal-title":"J. Med. Biochem."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"619","DOI":"10.2144\/000112776","article-title":"Twenty-five years of quantitative PCR for gene expression analysis","volume":"44","author":"VanGuilder","year":"2008","journal-title":"BioTechniques"},{"key":"ref_28","doi-asserted-by":"crossref","unstructured":"Lowe, R., Shirley, N., Bleackley, M., Dolan, S., and Shafee, T. (2017). Transcriptomics technologies. PLoS Comput. Biol., 13.","DOI":"10.1371\/journal.pcbi.1005457"},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"611","DOI":"10.1373\/clinchem.2008.112797","article-title":"The MIQE Guidelines: Minimum Information for Publication of Quantitative Real-Time PCR Experiments","volume":"55","author":"Bustin","year":"2009","journal-title":"Clin. Chem."},{"key":"ref_30","first-page":"46","article-title":"The State of RT-Quantitative PCR: Firsthand Observations of Implementation of Minimum Information for the Publication of Quantitative Real-Time PCR Experiments (MIQE)","volume":"24","author":"Taylor","year":"2013","journal-title":"J. Mol. Microbiol. Biotechnol."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Chapman, J.R., and Waldenstr\u00f6m, J. (2015). With Reference to Reference Genes: A Systematic Review of Endogenous Controls in Gene Expression Studies. PLoS ONE, 10.","DOI":"10.1371\/journal.pone.0141853"},{"key":"ref_32","doi-asserted-by":"crossref","unstructured":"Riedel, G., R\u00fcdrich, U., Fekete-Drimusz, N., Manns, M.P., Vondran, F.W.R., and Bock, M. (2014). An Extended \u0394CT-Method Facilitating Normalisation with Multiple Reference Genes Suited for Quantitative RT-PCR Analyses of Human Hepatocyte-Like Cells. PLoS ONE, 9.","DOI":"10.1371\/journal.pone.0093031"},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"389","DOI":"10.1152\/physiolgenomics.00025.2005","article-title":"GAPDH as a housekeeping gene: Analysis of GAPDH mRNA expression in a panel of 72 human tissues","volume":"21","author":"Barber","year":"2005","journal-title":"Physiol. Genom."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"307","DOI":"10.1006\/mcpr.2001.0376","article-title":"\u03b2-Actin\u2014An unsuitable internal control for RT-PCR","volume":"15","author":"Selvey","year":"2001","journal-title":"Mol. Cell. Probes"},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1006\/abio.2000.4889","article-title":"Unsuitability of Using Ribosomal RNA as Loading Control for Northern Blot Analyses Related to the Imbalance between Messenger and Ribosomal RNA Content in Rat Mammary Tumors","volume":"288","author":"Solanas","year":"2001","journal-title":"Anal. Biochem."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/S0168-1656(99)00163-7","article-title":"Housekeeping genes as internal standards: Use and limits","volume":"75","author":"Thellin","year":"1999","journal-title":"J. Biotechnol."},{"key":"ref_37","doi-asserted-by":"crossref","unstructured":"Silver, N., Best, S., Jiang, J., and Thein, S.L. (2006). Selection of housekeeping genes for gene expression studies in human reticulocytes using real-time PCR. BMC Mol. Biol., 7.","DOI":"10.1186\/1471-2199-7-33"},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"research0034","DOI":"10.1186\/gb-2002-3-7-research0034","article-title":"Accurate normalization of real-time quantitative RT-PCR data by geometric averaging of multiple internal control genes","volume":"3","author":"Vandesompele","year":"2002","journal-title":"Genome Biol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"509","DOI":"10.1023\/B:BILE.0000019559.84305.47","article-title":"Determination of stable housekeeping genes, differentially regulated target genes and sample integrity: BestKeeper\u2014Excel-based tool using pair-wise correlations","volume":"26","author":"Pfaffl","year":"2004","journal-title":"Biotechnol. Lett."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"5245","DOI":"10.1158\/0008-5472.CAN-04-0496","article-title":"Normalization of Real-Time Quantitative Reverse Transcription-PCR Data: A Model-Based Variance Estimation Approach to Identify Genes Suited for Normalization, Applied to Bladder and Colon Cancer Data Sets","volume":"64","author":"Andersen","year":"2004","journal-title":"Cancer Res."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"37116","DOI":"10.1038\/srep37116","article-title":"Assessment of brain reference genes for RT-qPCR studies in neurodegenerative diseases","volume":"6","author":"Rydbirk","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"39","DOI":"10.1002\/dneu.22558","article-title":"Identifying suitable reference genes for developing and injured mouse CNS tissues","volume":"78","author":"Xu","year":"2017","journal-title":"Dev. Neurobiol."},{"key":"ref_43","doi-asserted-by":"crossref","unstructured":"Schwarz, A.P., Kovalenko, A.A., Malygina, D.A., Postnikova, T.Y., Zubareva, O.E., and Zaitsev, A.V. (2020). Reference Gene Validation in the Brain Regions of Young Rats after Pentylenetetrazole-Induced Seizures. Biomedicines, 8.","DOI":"10.3390\/biomedicines8080239"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"3278","DOI":"10.1038\/s41598-021-82800-5","article-title":"Assessment of common housekeeping genes as reference for gene expression studies using RT-qPCR in mouse choroid plexus","volume":"11","author":"Ho","year":"2021","journal-title":"Sci. Rep."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"msab348","DOI":"10.1093\/molbev\/msab348","article-title":"Phylogenetic and Molecular Analyses Identify SNORD116 Targets Involved in the Prader\u2013Willi Syndrome","volume":"39","author":"Baldini","year":"2021","journal-title":"Mol. Biol. Evol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"275","DOI":"10.1038\/s41467-021-27781-9","article-title":"Mammalian brain glycoproteins exhibit diminished glycan complexity compared to other tissues","volume":"13","author":"Williams","year":"2022","journal-title":"Nat. Commun."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1261\/rna.1755810","article-title":"Application of housekeeping npcRNAs for quantitative expression analysis of human transcriptome by real-time PCR","volume":"16","author":"Galiveti","year":"2009","journal-title":"RNA"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"375","DOI":"10.3389\/fncel.2015.00375","article-title":"Bypassing hazard of housekeeping genes: Their evaluation in rat granule neurons treated with cerebrospinal fluid of multiple sclerosis subjects","volume":"9","author":"Mathur","year":"2015","journal-title":"Front. Cell. Neurosci."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"75","DOI":"10.1007\/s11103-012-9885-2","article-title":"miRDeepFinder: A miRNA analysis tool for deep sequencing of plant small RNAs","volume":"80","author":"Xie","year":"2012","journal-title":"Plant Mol. Biol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"1101","DOI":"10.1093\/hmg\/ddab103","article-title":"Snord116 Post-transcriptionally Increases Nhlh2 mRNA Stability: Implications for Human Prader-Willi Syndrome","volume":"30","author":"Kocher","year":"2021","journal-title":"Hum. Mol. Genet."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"749164","DOI":"10.3389\/fnmol.2021.749164","article-title":"Neuroligin-3: A Circuit-Specific Synapse Organizer That Shapes Normal Function and Autism Spectrum Disorder-Associated Dysfunction","volume":"14","author":"Uchigashima","year":"2021","journal-title":"Front. Mol. Neurosci."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"8589","DOI":"10.1074\/jbc.AC120.012571","article-title":"Neuroligin3 splice isoforms shape inhibitory synaptic function in the mouse hippocampus","volume":"295","author":"Uchigashima","year":"2020","journal-title":"J. Biol. Chem."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"118","DOI":"10.1186\/s13023-017-0673-6","article-title":"Early diagnosis and care is achieved but should be improved in infants with Prader-Willi syndrome","volume":"12","author":"Bar","year":"2017","journal-title":"Orphanet J. Rare Dis."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"E1072","DOI":"10.1210\/jc.2012-3888","article-title":"Growth Hormone Research Society Workshop Summary: Consensus Guidelines for Recombinant Human Growth Hormone Therapy in Prader-Willi Syndrome","volume":"98","author":"Deal","year":"2013","journal-title":"J. Clin. Endocrinol. Metab."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"ra92","DOI":"10.1126\/scisignal.2003184","article-title":"IGFBP7 Binds to the IGF-1 Receptor and Blocks Its Activation by Insulin-Like Growth Factors","volume":"5","author":"Evdokimova","year":"2012","journal-title":"Sci. Signal."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"612","DOI":"10.1016\/j.bbrc.2003.09.058","article-title":"Proteolytic processing of IGFBP-related protein-1 (TAF\/angiomodulin\/mac25) modulates its biological activity","volume":"310","author":"Ahmed","year":"2003","journal-title":"Biochem. Biophys. Res. Commun."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"2313","DOI":"10.1016\/j.febslet.2012.06.037","article-title":"IGFBP7\u2019s susceptibility to proteolysis is altered by A-to-I RNA editing of its transcript","volume":"586","author":"Sie","year":"2012","journal-title":"FEBS Lett."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"727","DOI":"10.3389\/fonc.2020.00727","article-title":"Insulin Growth Factor Binding Protein 7 (IGFBP7)-Related Cancer and IGFBP3 and IGFBP7 Crosstalk","volume":"10","author":"Jin","year":"2020","journal-title":"Front. Oncol."},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Ding, F., Li, H.H., Zhang, S., Solomon, N.M., Camper, S., Cohen, P., and Francke, U. (2008). SnoRNA Snord116 (Pwcr1\/MBII-85) Deletion Causes Growth Deficiency and Hyperphagia in Mice. PLoS ONE, 3.","DOI":"10.1371\/journal.pone.0001709"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"347","DOI":"10.1210\/er.2015-1117","article-title":"PCSK1 Mutations and Human Endocrinopathies: From Obesity to Gastrointestinal Disorders","volume":"37","author":"Stijnen","year":"2016","journal-title":"Endocr. Rev."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"10293","DOI":"10.1073\/pnas.162352599","article-title":"Disruption of PC1\/3 expression in mice causes dwarfism and multiple neuroendocrine peptide processing defects","volume":"99","author":"Zhu","year":"2002","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"e12994","DOI":"10.1111\/jne.12994","article-title":"Hypothalamic neuropeptides and neurocircuitries in Prader Willi syndrome","volume":"33","author":"Fliers","year":"2021","journal-title":"J. Neuroendocr."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"14380","DOI":"10.1038\/srep14380","article-title":"Genetic polymorphisms of PCSK2 are associated with glucose homeostasis and progression to type 2 diabetes in a Chinese population","volume":"5","author":"Chang","year":"2015","journal-title":"Sci. Rep."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"3245","DOI":"10.1007\/s00125-012-2728-5","article-title":"Effect of a common variant of the PCSK2 gene on reduced insulin secretion","volume":"55","author":"Jonsson","year":"2012","journal-title":"Diabetologia"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1016\/j.ymgme.2007.05.014","article-title":"Association of the proprotein convertase subtilisin\/kexin-type 2 (PCSK2) gene with type 2 diabetes in an African American population","volume":"92","author":"Leak","year":"2007","journal-title":"Mol. Genet. Metab."},{"key":"ref_66","doi-asserted-by":"crossref","unstructured":"Clerc, A., Coupaye, M., Mosbah, H., Pinto, G., Laurier, V., Mourre, F., Merrien, C., Diene, G., Poitou, C., and Tauber, M. (2021). Diabetes Mellitus in Prader-Willi Syndrome: Natural History during the Transition from Childhood to Adulthood in a Cohort of 39 Patients. J. Clin. Med., 10.","DOI":"10.3390\/jcm10225310"},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"4227","DOI":"10.1093\/hmg\/ddp373","article-title":"Imprinting regulates mammalian snoRNA-encoding chromatin decondensation and neuronal nucleolar size","volume":"18","author":"Leung","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_68","doi-asserted-by":"crossref","first-page":"70","DOI":"10.1242\/jcs.054957","article-title":"Long nuclear-retained non-coding RNAs and allele-specific higher-order chromatin organization at imprinted snoRNA gene arrays","volume":"123","author":"Vitali","year":"2010","journal-title":"J. Cell Sci."},{"key":"ref_69","doi-asserted-by":"crossref","unstructured":"Knott, B., Kocher, M.A., Paz, H.A., Hamm, S.E., Fink, W., Mason, J., Grange, R.W., Wankhade, U.D., and Good, D.J. (2022). Dietary Conjugated Linoleic Acid Reduces Body Weight and Fat in Snord116m+\/p\u2212 and Snord116m\u2212\/p\u2212 Mouse Models of Prader\u2013Willi Syndrome. Nutrients, 14.","DOI":"10.3390\/nu14040860"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"960","DOI":"10.1172\/JCI97007","article-title":"Hypothalamic loss of Snord116 recapitulates the hyperphagia of Prader-Willi syndrome","volume":"128","author":"Lam","year":"2018","journal-title":"J. Clin. Investig."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1038\/ng1136","article-title":"Mutations of the X-linked genes encoding neuroligins NLGN3 and NLGN4 are associated with autism","volume":"34","author":"Jamain","year":"2003","journal-title":"Nat. Genet."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1126\/science.1146221","article-title":"A Neuroligin-3 Mutation Implicated in Autism Increases Inhibitory Synaptic Transmission in Mice","volume":"318","author":"Tabuchi","year":"2007","journal-title":"Science"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1016\/j.nbd.2018.09.017","article-title":"Inactivation of Magel2 suppresses oxytocin neurons through synaptic excitation-inhibition imbalance","volume":"121","author":"Ates","year":"2018","journal-title":"Neurobiol. Dis."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"3101","DOI":"10.1093\/hmg\/9.20.3101","article-title":"Disruption of the mouse Necdin gene results in hypothalamic and behavioral alterations reminiscent of the human Prader-Willi syndrome","volume":"9","author":"Muscatelli","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"20398","DOI":"10.1038\/srep20398","article-title":"Maternal transcription of non-protein coding RNAs from the PWS-critical region rescues growth retardation in mice","volume":"6","author":"Rozhdestvensky","year":"2016","journal-title":"Sci. Rep."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"230","DOI":"10.1126\/science.1118265","article-title":"The snoRNA HBII-52 Regulates Alternative Splicing of the Serotonin Receptor 2C","volume":"311","author":"Kishore","year":"2006","journal-title":"Science"},{"key":"ref_77","unstructured":"Brosius, J., H\u00fcttenhofer, A., and Tiedge, H. (2003). Brain-Specific Nonmessenger RNAs. Non-Coding RNAs: Molecular Biology and Molecular Medicine, Springer Science & Business Media."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"2140","DOI":"10.1093\/hmg\/ddp137","article-title":"Loss of the imprinted snoRNA mbii-52 leads to increased 5htr2c pre-RNA editing and altered 5HT2CR-mediated behaviour","volume":"18","author":"Doe","year":"2009","journal-title":"Hum. Mol. Genet."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1093\/gbe\/evt155","article-title":"Alternative Processing as Evolutionary Mechanism for the Origin of Novel Nonprotein Coding RNAs","volume":"5","author":"Mo","year":"2013","journal-title":"Genome Biol. Evol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"3401","DOI":"10.1016\/j.celrep.2018.03.018","article-title":"A Transcriptomic Signature of the Hypothalamic Response to Fasting and BDNF Deficiency in Prader-Willi Syndrome","volume":"22","author":"Bochukova","year":"2018","journal-title":"Cell Rep."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"4300","DOI":"10.1038\/s41598-019-39940-6","article-title":"Ectopic expression of Snord115 in choroid plexus interferes with editing but not splicing of 5-Ht2c receptor pre-mRNA in mice","volume":"9","author":"Raabe","year":"2019","journal-title":"Sci. Rep."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"e60862","DOI":"10.7554\/eLife.60862","article-title":"Reassessment of the involvement of Snord115 in the serotonin 2c receptor pathway in a genetically relevant mouse model","volume":"9","author":"Hebras","year":"2020","journal-title":"eLife"},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"172","DOI":"10.1016\/j.gene.2007.10.037","article-title":"snoTARGET shows that human orphan snoRNA targets locate close to alternative splice junctions","volume":"408","author":"Bazeley","year":"2008","journal-title":"Gene"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"2242","DOI":"10.1093\/nar\/gkv075","article-title":"Yeast Kre33 and human NAT10 are conserved 18S rRNA cytosine acetyltransferases that modify tRNAs assisted by the adaptor Tan1\/THUMPD1","volume":"43","author":"Sharma","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"6284","DOI":"10.1093\/nar\/gkac404","article-title":"Probing small ribosomal subunit RNA helix 45 acetylation across eukaryotic evolution","volume":"50","author":"Quillien","year":"2022","journal-title":"Nucleic Acids Res."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1093\/molbev\/mst209","article-title":"Matching of Soulmates: Coevolution of snoRNAs and Their Targets","volume":"31","author":"Kehr","year":"2013","journal-title":"Mol. Biol. Evol."},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"3401","DOI":"10.1038\/s41467-019-11375-7","article-title":"Modification of messenger RNA by 2\u2032-O-methylation regulates gene expression in vivo","volume":"10","author":"Elliott","year":"2019","journal-title":"Nat. Commun."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1083\/jcb.200411129","article-title":"ADAR2-mediated editing of RNA substrates in the nucleolus is inhibited by C\/D small nucleolar RNAs","volume":"169","author":"Vitali","year":"2005","journal-title":"J. Cell Biol."}],"container-title":["International Journal of Molecular Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/15\/8729\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T00:04:41Z","timestamp":1760141081000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/1422-0067\/23\/15\/8729"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,8,5]]},"references-count":88,"journal-issue":{"issue":"15","published-online":{"date-parts":[[2022,8]]}},"alternative-id":["ijms23158729"],"URL":"https:\/\/doi.org\/10.3390\/ijms23158729","relation":{},"ISSN":["1422-0067"],"issn-type":[{"value":"1422-0067","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,8,5]]}}}