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An in vitro model of acute horizontal basal cell activation reveals gene regulatory networks underlying the nascent activation phase. 急性水平基底细胞活化的体外模型揭示了新生活化阶段的基因调控网络。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-08-13 Epub Date: 2024-07-25 DOI: 10.1016/j.stemcr.2024.06.011
Camila M Barrios-Camacho, Matthew J Zunitch, Jonathan D Louie, Woochan Jang, James E Schwob
{"title":"An in vitro model of acute horizontal basal cell activation reveals gene regulatory networks underlying the nascent activation phase.","authors":"Camila M Barrios-Camacho, Matthew J Zunitch, Jonathan D Louie, Woochan Jang, James E Schwob","doi":"10.1016/j.stemcr.2024.06.011","DOIUrl":"10.1016/j.stemcr.2024.06.011","url":null,"abstract":"<p><p>While horizontal basal cells (HBCs) make minor contributions to olfactory epithelium (OE) regeneration during homeostatic conditions, they possess a potent, latent capacity to activate and subsequently regenerate the OE following severe injury. Activation requires, and is mediated by, the downregulation of the transcription factor (TF) TP63. In this paper, we describe the cellular processes that drive the nascent stages of HBC activation. The compound phorbol 12-myristate 13-acetate (PMA) induces a rapid loss in TP63 protein and rapid enrichment of HOPX and the nuclear translocation of RELA, previously identified as components of HBC activation. Using bulk RNA sequencing (RNA-seq), we find that PMA-treated HBCs pass through various stages of activation identifiable by transcriptional regulatory signatures that mimic stages identified in vivo. These temporal stages are associated with varying degrees of engraftment and differentiation potential in transplantation assays. Together, these data show that our in vitro HBC activation system models physiologically relevant features of in vivo HBC activation and identifies new candidates for mechanistic testing.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"1156-1171"},"PeriodicalIF":5.9,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11368683/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141767470","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Retinal cells derived from patients with DRAM2-dependent CORD21 dystrophy exhibit key lysosomal enzyme deficiency and lysosomal content accumulation. DRAM2依赖性CORD21营养不良症患者的视网膜细胞表现出关键溶酶体酶缺乏和溶酶体内容物积累。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-08-13 Epub Date: 2024-07-03 DOI: 10.1016/j.stemcr.2024.06.002
Rozaliya Tsikandelova, Eldo Galo, Edvinas Cerniauskas, Dean Hallam, Maria Georgiou, Rodrigo Cerna-Chavez, Robert Atkinson, Pavel Palmowski, Florence Burté, Tracey Davies, David H Steel, Martin McKibbin, Jacquelyn Bond, Jennifer Haggarty, Phil Whitfield, Viktor Korolchuk, Lyle Armstrong, Chunbo Yang, Birthe Dorgau, Marzena Kurzawa-Akanbi, Majlinda Lako
{"title":"Retinal cells derived from patients with DRAM2-dependent CORD21 dystrophy exhibit key lysosomal enzyme deficiency and lysosomal content accumulation.","authors":"Rozaliya Tsikandelova, Eldo Galo, Edvinas Cerniauskas, Dean Hallam, Maria Georgiou, Rodrigo Cerna-Chavez, Robert Atkinson, Pavel Palmowski, Florence Burté, Tracey Davies, David H Steel, Martin McKibbin, Jacquelyn Bond, Jennifer Haggarty, Phil Whitfield, Viktor Korolchuk, Lyle Armstrong, Chunbo Yang, Birthe Dorgau, Marzena Kurzawa-Akanbi, Majlinda Lako","doi":"10.1016/j.stemcr.2024.06.002","DOIUrl":"10.1016/j.stemcr.2024.06.002","url":null,"abstract":"<p><p>Biallelic mutations in DRAM2 lead to an autosomal recessive cone-rod dystrophy known as CORD21, which typically presents between the third and sixth decades of life. Although DRAM2 localizes to the lysosomes of photoreceptor and retinal pigment epithelium (RPE) cells, its specific role in retinal degeneration has not been fully elucidated. In this study, we generated and characterized retinal organoids (ROs) and RPE cells from induced pluripotent stem cells (iPSCs) derived from two CORD21 patients. Our investigation revealed that CORD21-ROs and RPE cells exhibit abnormalities in lipid metabolism, defects in autophagic flux, accumulation of aberrant lysosomal content, and reduced lysosomal enzyme activity. We identified potential interactions of DRAM2 with vesicular trafficking proteins, suggesting its involvement in this cellular process. These findings collectively suggest that DRAM2 plays a crucial role in maintaining the integrity of photoreceptors and RPE cells by regulating lysosomal function, autophagy, and potentially vesicular trafficking.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"1107-1121"},"PeriodicalIF":5.9,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11368688/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141535315","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A dual-color PAX7 and MYF5 in vivo reporter to investigate muscle stem cell heterogeneity in regeneration and aging. 双色 PAX7 和 MYF5 体内报告器,用于研究再生和衰老过程中肌肉干细胞的异质性。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-13 DOI: 10.1016/j.stemcr.2024.05.005
Sara Ancel, Joris Michaud, Federico Sizzano, Loic Tauzin, Manuel Oliveira, Eugenia Migliavacca, Svenja C Schüler, Sruthi Raja, Gabriele Dammone, Sonia Karaz, José L Sánchez-García, Sylviane Metairon, Guillaume Jacot, C Florian Bentzinger, Jérôme N Feige, Pascal Stuelsatz
{"title":"A dual-color PAX7 and MYF5 in vivo reporter to investigate muscle stem cell heterogeneity in regeneration and aging.","authors":"Sara Ancel, Joris Michaud, Federico Sizzano, Loic Tauzin, Manuel Oliveira, Eugenia Migliavacca, Svenja C Schüler, Sruthi Raja, Gabriele Dammone, Sonia Karaz, José L Sánchez-García, Sylviane Metairon, Guillaume Jacot, C Florian Bentzinger, Jérôme N Feige, Pascal Stuelsatz","doi":"10.1016/j.stemcr.2024.05.005","DOIUrl":"10.1016/j.stemcr.2024.05.005","url":null,"abstract":"<p><p>Increasing evidence suggests that the muscle stem cell (MuSC) pool is heterogeneous. In particular, a rare subset of PAX7-positive MuSCs that has never expressed the myogenic regulatory factor MYF5 displays unique self-renewal and engraftment characteristics. However, the scarcity and limited availability of protein markers make the characterization of these cells challenging. Here, we describe the generation of StemRep reporter mice enabling the monitoring of PAX7 and MYF5 proteins based on equimolar levels of dual nuclear fluorescence. High levels of PAX7 protein and low levels of MYF5 delineate a deeply quiescent MuSC subpopulation with an increased capacity for asymmetric division and distinct dynamics of activation, proliferation, and commitment. Aging primarily reduces the MYF5<sup>Low</sup> MuSCs and skews the stem cell pool toward MYF5<sup>High</sup> cells with lower quiescence and self-renewal potential. Altogether, we establish the StemRep model as a versatile tool to study MuSC heterogeneity and broaden our understanding of mechanisms regulating MuSC quiescence and self-renewal in homeostatic, regenerating, and aged muscles.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"1024-1040"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252486/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321676","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Anticipating in vitro gametogenesis: Hopes and concerns for IVG among diverse stakeholders. 预期体外配子生成:不同利益相关者对体外配子生成的希望和担忧。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-06 DOI: 10.1016/j.stemcr.2024.05.002
Anne Le Goff, Robbin Jeffries Hein, Ariel N Hart, Isaias Roberson, Hannah L Landecker
{"title":"Anticipating in vitro gametogenesis: Hopes and concerns for IVG among diverse stakeholders.","authors":"Anne Le Goff, Robbin Jeffries Hein, Ariel N Hart, Isaias Roberson, Hannah L Landecker","doi":"10.1016/j.stemcr.2024.05.002","DOIUrl":"10.1016/j.stemcr.2024.05.002","url":null,"abstract":"<p><p>In vitro gametogenesis (IVG), the reconstitution of germ cell development in vitro, is an emerging stem cell-based technology with profound implications for reproductive science. Despite researchers' long-term goals for future clinical applications, little is currently known about the views of IVG held by the stakeholders potentially most affected by its introduction in humans. We conducted focus groups and interviews with 80 individuals with lived experience of infertility and/or LGBTQ+ family formation in the US, two intersecting groups of potential IVG users. Respondents expressed hope that IVG would lead to higher reproductive success than current assisted reproductive technology (ART), alleviate suffering associated with ART use, and promote greater social inclusion, while expressing concerns predominantly framed in terms of equity and safety. These findings underscore the importance of sustained engagement with stakeholders with relevant experience to anticipate the implications of IVG for research and clinical translation.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"933-945"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252480/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141288691","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Self-assembling 3D vessel-on-chip model with hiPSC-derived astrocytes. 利用源自 hiPSC 的星形胶质细胞自组装三维片上血管模型。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-13 DOI: 10.1016/j.stemcr.2024.05.006
Dennis M Nahon, Marc Vila Cuenca, Francijna E van den Hil, Michel Hu, Tessa de Korte, Jean-Philippe Frimat, Arn M J M van den Maagdenberg, Christine L Mummery, Valeria V Orlova
{"title":"Self-assembling 3D vessel-on-chip model with hiPSC-derived astrocytes.","authors":"Dennis M Nahon, Marc Vila Cuenca, Francijna E van den Hil, Michel Hu, Tessa de Korte, Jean-Philippe Frimat, Arn M J M van den Maagdenberg, Christine L Mummery, Valeria V Orlova","doi":"10.1016/j.stemcr.2024.05.006","DOIUrl":"10.1016/j.stemcr.2024.05.006","url":null,"abstract":"<p><p>Functionality of the blood-brain barrier (BBB) relies on the interaction between endothelial cells (ECs), pericytes, and astrocytes to regulate molecule transport within the central nervous system. Most experimental models for the BBB rely on freshly isolated primary brain cells. Here, we explored human induced pluripotent stem cells (hiPSCs) as a cellular source for astrocytes in a 3D vessel-on-chip (VoC) model. Self-organized microvascular networks were formed by combining hiPSC-derived ECs, human brain vascular pericytes, and hiPSC-derived astrocytes within a fibrin hydrogel. The hiPSC-ECs and pericytes showed close interactions, but, somewhat unexpectedly, addition of astrocytes disrupted microvascular network formation. However, continuous fluid perfusion or activation of cyclic AMP (cAMP) signaling rescued the vascular organization and decreased vascular permeability. Nevertheless, astrocytes did not affect the expression of proteins related to junction formation, transport, or extracellular matrix, indicating that, despite other claims, hiPSC-derived ECs do not entirely acquire a BBB-like identity in the 3D VoC model.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"946-956"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252484/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321678","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
O-GlcNAcase regulates pluripotency states of human embryonic stem cells. O-GlcNA酶调节人类胚胎干细胞的多能状态。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-27 DOI: 10.1016/j.stemcr.2024.05.009
Qianyu Liu, Cheng Chen, Zhiya Fan, Honghai Song, Yutong Sha, Liyang Yu, Yingjie Wang, Weijie Qin, Wen Yi
{"title":"O-GlcNAcase regulates pluripotency states of human embryonic stem cells.","authors":"Qianyu Liu, Cheng Chen, Zhiya Fan, Honghai Song, Yutong Sha, Liyang Yu, Yingjie Wang, Weijie Qin, Wen Yi","doi":"10.1016/j.stemcr.2024.05.009","DOIUrl":"10.1016/j.stemcr.2024.05.009","url":null,"abstract":"<p><p>Understanding the regulation of human embryonic stem cells (hESCs) pluripotency is critical to advance the field of developmental biology and regenerative medicine. Despite the recent progress, molecular events regulating hESC pluripotency, especially the transition between naive and primed states, still remain unclear. Here we show that naive hESCs display lower levels of O-linked N-acetylglucosamine (O-GlcNAcylation) than primed hESCs. O-GlcNAcase (OGA), the key enzyme catalyzing the removal of O-GlcNAc from proteins, is highly expressed in naive hESCs and is important for naive pluripotency. Depletion of OGA accelerates naive-to-primed pluripotency transition. OGA is transcriptionally regulated by EP300 and acts as a transcription regulator of genes important for maintaining naive pluripotency. Moreover, we profile protein O-GlcNAcylation of the two pluripotency states by quantitative proteomics. Together, this study identifies OGA as an important factor of naive pluripotency in hESCs and suggests that O-GlcNAcylation has a broad effect on hESCs homeostasis.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"993-1009"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252487/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141470727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Deciphering the heterogeneity of differentiating hPSC-derived corneal limbal stem cells through single-cell RNA sequencing. 通过单细胞 RNA 测序破解分化的 hPSC 衍生角膜缘干细胞的异质性。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-27 DOI: 10.1016/j.stemcr.2024.06.001
Meri Vattulainen, Jos G A Smits, Julian A Arts, Dulce Lima Cunha, Tanja Ilmarinen, Heli Skottman, Huiqing Zhou
{"title":"Deciphering the heterogeneity of differentiating hPSC-derived corneal limbal stem cells through single-cell RNA sequencing.","authors":"Meri Vattulainen, Jos G A Smits, Julian A Arts, Dulce Lima Cunha, Tanja Ilmarinen, Heli Skottman, Huiqing Zhou","doi":"10.1016/j.stemcr.2024.06.001","DOIUrl":"10.1016/j.stemcr.2024.06.001","url":null,"abstract":"<p><p>A comprehensive understanding of the human pluripotent stem cell (hPSC) differentiation process stands as a prerequisite for the development of hPSC-based therapeutics. In this study, single-cell RNA sequencing (scRNA-seq) was performed to decipher the heterogeneity during differentiation of three hPSC lines toward corneal limbal stem cells (LSCs). The scRNA-seq data revealed nine clusters encompassing the entire differentiation process, among which five followed the anticipated differentiation path of LSCs. The remaining four clusters were previously undescribed cell states that were annotated as either mesodermal-like or undifferentiated subpopulations, and their prevalence was hPSC line dependent. Distinct cluster-specific marker genes identified in this study were confirmed by immunofluorescence analysis and employed to purify hPSC-derived LSCs, which effectively minimized the variation in the line-dependent differentiation efficiency. In summary, scRNA-seq offered molecular insights into the heterogeneity of hPSC-LSC differentiation, allowing a data-driven strategy for consistent and robust generation of LSCs, essential for future advancement toward clinical translation.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"1010-1023"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252539/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141470726","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
TGF-β modulates cell fate in human ES cell-derived foregut endoderm by inhibiting Wnt and BMP signaling. TGF-β 通过抑制 Wnt 和 BMP 信号调节人 ES 细胞衍生的前肠内胚层细胞命运。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-27 DOI: 10.1016/j.stemcr.2024.05.010
Nina Sofi Funa, Heidi Katharina Mjoseng, Kristian Honnens de Lichtenberg, Silvia Raineri, Deniz Esen, Anuska la Rosa Egeskov-Madsen, Roberto Quaranta, Mette Christine Jørgensen, Maria Skjøtt Hansen, Jonas van Cuyl Kuylenstierna, Kim Bak Jensen, Yi Miao, K Christopher Garcia, Philip A Seymour, Palle Serup
{"title":"TGF-β modulates cell fate in human ES cell-derived foregut endoderm by inhibiting Wnt and BMP signaling.","authors":"Nina Sofi Funa, Heidi Katharina Mjoseng, Kristian Honnens de Lichtenberg, Silvia Raineri, Deniz Esen, Anuska la Rosa Egeskov-Madsen, Roberto Quaranta, Mette Christine Jørgensen, Maria Skjøtt Hansen, Jonas van Cuyl Kuylenstierna, Kim Bak Jensen, Yi Miao, K Christopher Garcia, Philip A Seymour, Palle Serup","doi":"10.1016/j.stemcr.2024.05.010","DOIUrl":"10.1016/j.stemcr.2024.05.010","url":null,"abstract":"<p><p>Genetic differences between pluripotent stem cell lines cause variable activity of extracellular signaling pathways, limiting reproducibility of directed differentiation protocols. Here we used human embryonic stem cells (hESCs) to interrogate how exogenous factors modulate endogenous signaling events during specification of foregut endoderm lineages. We find that transforming growth factor β1 (TGF-β1) activates a putative human OTX2/LHX1 gene regulatory network which promotes anterior fate by antagonizing endogenous Wnt signaling. In contrast to Porcupine inhibition, TGF-β1 effects cannot be reversed by exogenous Wnt ligands, suggesting that induction of SHISA proteins and intracellular accumulation of Fzd receptors render TGF-β1-treated cells refractory to Wnt signaling. Subsequently, TGF-β1-mediated inhibition of BMP and Wnt signaling suppresses liver fate and promotes pancreas fate. Furthermore, combined TGF-β1 treatment and Wnt inhibition during pancreatic specification reproducibly and robustly enhance INSULIN<sup>+</sup> cell yield across hESC lines. This modification of widely used differentiation protocols will enhance pancreatic β cell yield for cell-based therapeutic applications.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"973-992"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252478/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141470728","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Cellular and axonal transport phenotypes due to the C9ORF72 HRE in iPSC motor and sensory neurons. iPSC 运动和感觉神经元中 C9ORF72 HRE 导致的细胞和轴突运输表型。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-09 Epub Date: 2024-06-13 DOI: 10.1016/j.stemcr.2024.05.008
Jakub Scaber, Iona Thomas-Wright, Alex J Clark, Yinyan Xu, Björn F Vahsen, Mireia Carcolé, Ruxandra Dafinca, Lucy Farrimond, Adrian M Isaacs, David L Bennett, Kevin Talbot
{"title":"Cellular and axonal transport phenotypes due to the C9ORF72 HRE in iPSC motor and sensory neurons.","authors":"Jakub Scaber, Iona Thomas-Wright, Alex J Clark, Yinyan Xu, Björn F Vahsen, Mireia Carcolé, Ruxandra Dafinca, Lucy Farrimond, Adrian M Isaacs, David L Bennett, Kevin Talbot","doi":"10.1016/j.stemcr.2024.05.008","DOIUrl":"10.1016/j.stemcr.2024.05.008","url":null,"abstract":"<p><p>Induced pluripotent stem cell (iPSC)-derived motor neurons (MNs) from patients with amyotrophic lateral sclerosis (ALS) and the C9ORF72 hexanucleotide repeat expansion (HRE) have multiple cellular phenotypes, but which of these accurately reflect the biology underlying the cell-specific vulnerability of ALS is uncertain. We therefore compared phenotypes due to the C9ORF72 HRE in MNs with sensory neurons (SNs), which are relatively spared in ALS. The iPSC models were able to partially reproduce the differential gene expression seen between adult SNs and MNs. We demonstrated that the typical hallmarks of C9ORF72-ALS, including RNA foci and dipeptide formation, as well as specific axonal transport defects, occurred equally in MNs and SNs, suggesting that these in vitro phenotypes are not sufficient to explain the cell-type selectivity of ALS in isolation.</p>","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":"957-972"},"PeriodicalIF":5.9,"publicationDate":"2024-07-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252479/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141321677","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Recommendations on fit-for-purpose criteria to establish quality management for microphysiological systems and for monitoring their reproducibility. 关于建立微观生理系统质量管理和监测其再现性的适用标准的建议。
IF 5.9 2区 医学
Stem Cell Reports Pub Date : 2024-07-02 DOI: 10.1016/j.stemcr.2024.06.007
David Pamies, Jason Ekert, Marie-Gabrielle Zurich, Olivier Frey, Sophie Werner, Monica Piergiovanni, Benjamin S Freedman, Adrian Kee Keong Teo, Hendrik Erfurth, Darwin R Reyes, Peter Loskill, Pelin Candarlioglu, Laura Suter-Dick, Shan Wang, Thomas Hartung, Sandra Coecke, Glyn N Stacey, Beren Atac Wagegg, Eva-Maria Dehne, Francesca Pistollato, Marcel Leist
{"title":"Recommendations on fit-for-purpose criteria to establish quality management for microphysiological systems and for monitoring their reproducibility.","authors":"David Pamies, Jason Ekert, Marie-Gabrielle Zurich, Olivier Frey, Sophie Werner, Monica Piergiovanni, Benjamin S Freedman, Adrian Kee Keong Teo, Hendrik Erfurth, Darwin R Reyes, Peter Loskill, Pelin Candarlioglu, Laura Suter-Dick, Shan Wang, Thomas Hartung, Sandra Coecke, Glyn N Stacey, Beren Atac Wagegg, Eva-Maria Dehne, Francesca Pistollato, Marcel Leist","doi":"10.1016/j.stemcr.2024.06.007","DOIUrl":"10.1016/j.stemcr.2024.06.007","url":null,"abstract":"","PeriodicalId":21885,"journal":{"name":"Stem Cell Reports","volume":" ","pages":""},"PeriodicalIF":5.9,"publicationDate":"2024-07-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11252524/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141498980","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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