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| 1 | Genes for RNA-binding proteins involved in neuralspecific functions and diseases are downregulated in Rubinstein-Taybi iNeurons显示文摘Taking advantage of the fast-growing knowledge of RNA-binding proteins(RBPs)we review the signature of downregulated genes for RBPs in the transcriptome of induced pluripotent stem cell neurons(iNeurons)modelling the neurodevelopmental Rubinstein Taybi Syndrome(RSTS)caused by mutations in the genes encoding CBP/p300 acetyltransferases.We discuss top and functionally connected downregulated genes sorted to“RNA processing”and“Ribonucleoprotein complex biogenesis”Gene Ontology clusters.The first set of downregulated RBPs includes members of hnRNHP(A1,A2B1,D,G,H2-H1,MAGOHB,PAPBC),core subunits of U small nuclear ribonucleoproteins and Serine-Arginine splicing regulators families,acting in precursor messenger RNA alternative splicing and processing.Consistent with literature findings on reduced transcript levels of serine/arginine repetitive matrix 4(SRRM4)protein,the main regulator of the neural-specific microexons splicing program upon depletion of Ep300 and Crebbp in mouse neurons,RSTS iNeurons show downregulated genes for proteins impacting this network.We link downregulated genes to neurological disorders including the new HNRNPH1-related intellectual disability syndrome with clinical overlap to RSTS.The set of downregulated genes for Ribosome biogenesis includes several components of ribosomal subunits and nucleolar proteins,such NOP58 and fibrillarin that form complexes with snoRNAs with a central role in guiding post-transcriptional modifications needed for rRNA maturation.These nucleolar proteins are“dual”players as fibrillarin is also required for epigenetic regulation of ribosomal genes and conversely NOP58-associated snoRNA levels are under the control of NOP58 interactor BMAL1,a transcriptional regulator of the circadian rhythm.Additional downregulated genes for“dual specificity”RBPs such as RUVBL1 and METTL1 highlight the links between chromatin and the RBP-ome and the contribution of perturbations in their cross-talk to RSTS.We underline the hub position of CBP/p300 in chromatin regulation,the impact of its defect on neurons’post-transcriptional regulation of gene expression and the potential use of epidrugs in therapeutics of RBP-caused neurodevelopmental disorders. | Lidia Larizza Luciano Calzari Valentina Alari Silvia Russo | 2022 | Neural Regeneration Research2022，17，1： | 2 |
| 2 | Increasing risk of relapse after treatment of Clostridium dif{icile colitis in Quebec, Canada显示文摘 | Pepin J Alary M E Valiquette L | 2005 | Clin Infect Dis2005，40，15： | 1 |
| 3 | Complementary Hypothesis Concerning the Community Sexually Transmitted Disease Mass Treatment Puzzle in Rakai,Uganda显示文摘 | BOILY M G LOWNDES C M ALARY M | 2000 | AIDS2000，14，16： | 1 |
| 4 | Metabolism of 4-hydroxy-nonenal,a cytotoxic product of lipid peroxidation,in rat precision-cut liver slices显示文摘 | Laurent A Perdu-Durand E Alary J | 2000 | Toxicol Lett2000，114，： | 1 |
| 5 | A method to determine the potential toxicity of smoke from burning polymers:Ⅲ显示文摘 | Caldwell D J Y Alarie | 1991 | Journal of fire sciences1991，9，： | 1 |
| 6 | A method to determine the potential toxicity of smoke from burning polymers: Ⅲ 显示文摘 | Caldwell D J Y Alarie | 1991 | Journal of fire science1991，，9： | 1 |
| 7 | Supplementation with calcium+vitamin D enhances the benefi cial effect of weight loss on plasma lipid and lipoprotein concentration显示文摘 | Major GC Alarie F Dore J | 2007 | Am J Clin Nutr2007，85，1： | 1 |
| 8 | A discus- sion support model for a regional dairy-pasture system with an example from Reunion island显示文摘 | NIDUMOLU U B LUBBERS M ALARY V | 2011 | Journal of Agricultural Science2011，149，： | 1 |
| 9 | Toxicity of fire smoke显示文摘 | Alarie Y | 2002 | Crit Rev Toxicol2002，32，4： | 1 |
| 10 | Increasing risk of relapse after treatment of Clostridium difficile colitis in Quebec,Canada显示文摘 | Pepin J Alary ME Valiquette L | 2005 | Clin Infect Dis2005，40，： | 1 |
| 11 | Toxicity of fire smoke显示文摘 | Alarie Y | 2002 | Crit Rev Toxicol2002，32，4： | 1 |
| 12 | Inhalation toxicity of carbon monoxide and hydrogen cyanide gases released during the thermal decomposition of polymers显示文摘 | Esposito F Alarie Y | 1988 | Journal of Fire Sciences1988，6，3： | 1 |
| 13 | Calcium plus Vitamin D supplementation and fat mass loss in female very low-calcium consum- ers: Potential link with a calcium-specific appetite control 显示文摘 | MAJOR GC ALARIE F DORE J | 2009 | Br J Nutr2009，101，5： | 1 |
| 14 | A new surface- enhanced Raman scattering substrate based on silver nanoparticles in sol-gel显示文摘 | Stokes DL Alarie JP Ananthanarayanan V | 1999 | Proc SPIE1999，30，12： | 1 |
| 15 | Optimal placement and sizing method to improve the voltage stability margin in a distribution system using distributed generation 显示文摘 | Alari R EI-Saadany E Atwa Y | 2013 | IEEE Transactions on Power Systems2013，28，1： | 1 |
| 16 | Association of hepatitis B virus infection with other sexually transmitted infections in homosexual men:Omega Study Group显示文摘 | Remis RS Dufour A Alary M | 2000 | Am J Public Health2000，90，： | 1 |
| 17 | Antibody-- based submicroion biosensor for benzopyrene DNA aduct显示文摘 | Alarie JP Vo- Dinh T | 1996 | Polycydic Aromat Compounds1996，8，： | 1 |
| 18 | Prevalence of Chlamydia trachomatis and Neisseria gonorrhoeae among atrisk women,Young sex workers,and streel routh attending community organizations in Queber city,Canada显示文摘 | Poulin C Alary M Bernier F | 2001 | Sex Transm Dis2001，28，8： | 1 |
| 19 | Antibody-based submicron biosensor for benzopyrene DNA adduct显示文摘 | Alarie J P Vo-Dinh T | 1996 | Polycydic Aromat Compounds1996，8，： | 1 |
| 20 | Repeated intrathecal injections ofdezocine produce antinociception without evidence for neurotoxicityin the rat:a study of morphometric evaluation of spinal cord histolo-gy显示文摘 | Svensson BA Alari L Post C | 1992 | Anesth Analg1992，7，3： | 1 |
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