Article
Cell Biology
Yan-Jiao Lu, Wen-Wen Yu, Meng-Meng Cui, Xian-Xian Yu, Huan-Lei Song, Mei-Rong Bai, Wen-Jie Wu, Bei-Lin Gu, Jun Wang, Wei Cai, Xun Chu
Summary: Common variants of DSCAM were associated with HSCR risk in the Han Chinese population, while common variants of BACE2 were not. Knockdown of both dscams and bace2 in zebrafish resulted in reduced enteric neurons in the hindgut, while overexpression of DSCAM and BACE2 did not affect neuron number in zebrafish hindgut.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Tamas Kovacs, Viktoria Halasy, Csongor Petho, Emoke Szocs, Adam Soos, David Dora, Pascal de Santa Barbara, Sandrine Faure, Rhian Stavely, Allan M. Goldstein, Nandor Nagy, Nicolas Pilon
Summary: The enteric nervous system (ENS) is derived from vagal neural crest cells and plays a crucial role in gastrointestinal development. This study shows that the avian ceca, located at the junction of the midgut and hindgut, are necessary for hindgut ENS development. It also highlights the importance of bone morphogenetic protein-4 (BMP4) and glial cell line-derived neurotrophic factor (GDNF) signaling pathways in the migration of enteric neural crest-derived cells (ENCDC) and the formation of enteric ganglia.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Article
Biochemistry & Molecular Biology
Ashoka Bandla, Ellie Melancon, Charlotte R. Taylor, Ann E. Davidson, Judith S. Eisen, Julia Ganz
Summary: The receptor tyrosine kinase Ret plays a crucial role in regulating enteric nervous system (ENS) development, particularly in progenitor cell proliferation, migration, and survival. This study introduced a new BAC transgenic line that expresses GFP under the ret promoter, allowing visualization of Ret expression in live zebrafish. The research contributes to characterizing the Ret signaling pathway in ENS development from early stages to adulthood.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Developmental Biology
Viktoria Halasy, Emoke Szocs, Adam Soos, Tamas Kovacs, Nora Pecsenye-Fejszak, Ryo Hotta, Allan M. Goldstein, Nandor Nagy
Summary: The enteric nervous system (ENS) and peripheral ganglia provide innervation to the gastrointestinal tract, but the molecular mechanisms controlling extrinsic nerve fiber growth are not well understood. This study investigates the role of CXCR4 and CXCL12 in the development of colorectal innervation. It is found that CXCR4 is expressed in nerve fibers arising from the nerve of Remak (NoR) and the pelvic plexus, while CXCL12 is localized to the hindgut and enteric ganglia. Overexpression of CXCL12 enhances axonal projections to the gut from the NoR, while CXCR4 inhibition disrupts nerve fiber extension.
Article
Developmental Biology
Nandor Nagy, Tamas Kovacs, Rhian Stavely, Viktoria Halasy, Adam Soos, Emoke Szocs, Ryo Hotta, Hannah Graham, Allan M. Goldstein
Summary: The migration and proliferation of ENCCs are crucial for ENS formation, with the ceca playing an important role in ENS development. The non-canonical Wnt signaling pathway regulates ENCC differentiation by inhibiting enteric neuronal differentiation.
Article
Developmental Biology
Rhian Stavely, Ryo Hotta, Richard A. Guyer, Nicole Picard, Ahmed A. Rahman, Meredith Omer, Adam Soos, Emoke Szocs, Jessica Mueller, Allan M. Goldstein, Nandor Nagy
Summary: The development of the enteric nervous system is dependent on the colonization of the intestine by enteric neural crest-derived cells (ENCDCs). ENCDCs at the wavefront, which are highly migratory and proliferative, exhibit distinct molecular characteristics compared to trailing ENCDCs. These wavefront ENCDCs show altered expression of genes associated with migration and lack expression of genes related to neuronal or glial maturation.
Article
Genetics & Heredity
Keqiang Liu, Ru Chen, Minzhong Zhang, Yiming Gong, Yong Wang, Wei Cai
Summary: Variants in the ERBB3 gene can lead to different disease phenotypes, including lethal congenital contracture syndrome and evident intestinal dysmotility. These findings help to better characterize the ERBB3-related phenotype spectrum, and reveal phenotypic variability caused by ERBB3 deficiency among different individuals.
Review
Biochemistry & Molecular Biology
Amy Marie Holland, Ana Carina Bon-Frauches, Daniel Keszthelyi, Veerle Melotte, Werend Boesmans
Summary: The enteric nervous system (ENS) plays a crucial role in regulating gastrointestinal function, and its dysfunction is closely linked to gastrointestinal diseases. Recent research indicates that the multifaceted connections between the ENS, intestinal epithelium, immune system, and gut microbiome are essential for maintaining gastrointestinal homeostasis. Therefore, the ENS plays a significant role in the pathogenesis of enteric neuropathies, inflammatory bowel diseases, and colorectal cancer.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2021)
Article
Oncology
Leticia Villalba-Benito, Daniel Lopez-Lopez, Ana Torroglosa, Carlos S. Casimiro-Soriguer, Berta Luzon-Toro, Raquel Maria Fernandez, Maria Jose Moya-Jimenez, Guillermo Antinolo, Joaquin Dopazo, Salud Borrego
Summary: This study reveals a decrease in global DNA methylation in HSCR patients in CpG context, along with a greater hypomethylation of DMRs. The downregulation of Methyltransferase 3b in EPCs from HSCR patients correlates with these findings. New potential susceptibility genes for HSCR have been identified through the comparative analysis of DMRs, highlighting the importance of an adequate methylation pattern for proper ENS development.
CLINICAL EPIGENETICS
(2021)
Review
Cell Biology
Kathy Nga-Chu Lui, Elly Sau-Wai Ngan
Summary: Hirschsprung disease is a complex congenital disorder caused by defects in the development of the enteric nervous system. The use of human induced pluripotent stem cells has provided new tools for understanding the disease and developing therapies. These stem cell-based models complement traditional genetic screens and bioinformatic approaches, and have potential applications in translational medicine and drug discovery.
Article
Biochemistry & Molecular Biology
Donghao Tian, Wenyao Xu, Weikang Pan, Baijun Zheng, Weili Yang, Wanying Jia, Yong Liu, Malgorzata A. Garstka, Ya Gao, Hui Yu
Summary: Fecal microbiota transplantation (FMT) can improve cell therapy for hypoganglionosis by enhancing short-chain fatty acid metabolism-induced MEK1/2 signaling.
Review
Multidisciplinary Sciences
R. Diposarosa, N. A. Bustam, Edhyana Sahiratmadja, P. S. Susanto, Y. Sribudiani
Summary: Hirschsprung's disease is a developmental disorder of the enteric nervous system derived from neural crest cells, and changes in gene expression may contribute to its pathogenesis. Deviant genetic and epigenetic patterns, such as DNA methylation, histone modification, and miRNA regulation, can play a role in the development of neurocristopathy. Specifically, epigenetic regulation of miRNA expression and its relationship to cellular interactions and gene activation through major pathways are discussed in the context of Hirschsprung's disease.
Review
Biochemistry & Molecular Biology
Yue Ji, Paul Kwong-Hang Tam, Clara Sze-Man Tang
Summary: The development of the enteric nervous system relies on the interaction between enteric neural crest cells and the neural stem cell niche. Defects in ENCCs may lead to ENS dysfunction. HSCR patients may have niche defects affecting ECM-cell interaction, suggesting the need for complementary therapies involving drugs and niche-based treatments.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Cell & Tissue Engineering
Rhian Stavely, Sukhada Bhave, Wing Lam N. Ho, Minhal Ahmed, Weikang Pan, Ahmed A. Rahman, Jessica Ulloa, Nicole Bousquet, Meredith Omer, Richard Guyer, Nandor Nagy, Allan M. Goldstein, Ryo Hotta
Summary: The interaction between ENSCs and EMCs plays a crucial role in the normal development of the enteric nervous system, with potential implications for treating Hirschsprung disease. EMCs enhance the expansion and neurosphere-forming ability of ENCDCs through paracrine factors, leading to functional neurons and successful transplantation in the HSCR model.
Article
Pediatrics
Jing Wang, Jun Xiao, Xinyao Meng, Xufeng Chu, Di Di Zhuansun, Bo Xiong, Jiexiong Feng
Summary: The study revealed that the expression of NOX5 is significantly decreased in the aganglionic segment of HSCR colon patients. In zebrafish, NOX5 expression seems to be enriched in the nervous system. However, knockdown of NOX5 did not affect the development of the enteric nervous system and gastrointestinal motility in zebrafish.
Article
Pediatrics
Ning Li, Qiao Bao, Jiyan Yuan, Xuefeng Zhou, Jiexiong Feng, Wen Zhang
Summary: The study compared the safety and efficacy of open transumbilical intussusception reduction (OTIR) and laparoscopic reduction (LAP) in children. The results showed differences in reduction rates, operative times, and surgery costs between OTIR and LAP, but both methods had similar rates of complications.
JOURNAL OF PEDIATRIC SURGERY
(2021)
Review
Neurosciences
Kang Wang, Weicheng Duan, Yijie Duan, Yuxin Yu, Xiuyi Chen, Yinhui Xu, Haihong Chen, Hongzhi Huang, Bo Xiong
Summary: In recent years, the rapid increase in cases of Autism Spectrum Disorder (ASD) has been linked to various genetic abnormalities. A study analyzing 2944 related research articles published between 1997 and 2018 found the United States to be a leading explorer in this field globally. The development of high-throughput technologies and bioinformatics has shifted research methods towards big data-based pipelines, with a focus on deciphering the role of de novo mutations in ASD and advocating for effective collaboration among research centers and scientists in different fields.
Article
Cell Biology
Xue Xia, Di Wang, Lei Yu, Bin Wang, Wenjing Wang, Chunlei Jiao, Xuyong Chen, Hongyi Zhang, Feng Chen, Jiexiong Feng
Summary: This study found a large number of M1 macrophage infiltration in the NEC intestine, with TNF-alpha upregulating the expression of miR-222 and inhibiting c-kit expression. Modulations of M1 macrophages, TNF-alpha, or miR-222 may be potential therapeutic targets for NEC treatment.
INFLAMMATION RESEARCH
(2021)
Article
Pediatrics
Jing Wang, Jun Xiao, Xinyao Meng, Xufeng Chu, Di Di Zhuansun, Bo Xiong, Jiexiong Feng
Summary: The study revealed that the expression of NOX5 is significantly decreased in the aganglionic segment of HSCR colon patients. In zebrafish, NOX5 expression seems to be enriched in the nervous system. However, knockdown of NOX5 did not affect the development of the enteric nervous system and gastrointestinal motility in zebrafish.
Article
Pediatrics
Feng Chen, Xiaoyu Wei, Xiaohua Chen, Lei Xiang, Jiexiong Feng
Summary: The study aims to compare laparoscopic-assisted reoperative surgery with transabdominal pull-through surgery in patients with Hirschsprung's disease and overflow fecal incontinence. Laparoscopic-assisted reoperation showed shorter operation time, less blood loss, shorter hospital stays, and lower post-operative complications compared to transabdominal pull-through surgery.
FRONTIERS IN PEDIATRICS
(2021)
Article
Oncology
Weicheng Duan, Kang Wang, Yijie Duan, Xiuyi Chen, Xufeng Chu, Ping Hu, Bo Xiong
Summary: This study successfully established a prognostic model for Malignant Pleural Mesothelioma (MPM) by comprehensively analyzing the ceRNA network of tumor cells. It identified genes like CDK6 and SGMS1-AS1 that play independent roles in the prognosis of MPM, and highlighted the significant roles of extracellular matrix components and cell division dysfunction in the invasion of MPM.
FRONTIERS IN ONCOLOGY
(2021)
Article
Pediatrics
Maggie L. Westfal, Ongoly Okiemy, Patrick Ho Yu Chung, Jiexiong Feng, Changgui Lu, Miyano Go, Paul Kwong Hang Tam, Weibing Tang, Kenneth Kak Yuen Wong, Atsuyuki Yamataka, Richard A. Guyer, Daniel P. Doody, Allan M. Goldstein
Summary: The study suggests that infants undergoing endorectal pull-through at age <2.5 months may have worse functional outcomes, including higher rates of soiling, anastomotic stricture, and anastomotic leak.
JOURNAL OF PEDIATRIC SURGERY
(2022)
Article
Chemistry, Physical
Ping Chen, Ping Wu, Xiao Wan, Qiong Wang, Chao Xu, Ming Yang, Jiexiong Feng, Bin Hu, Zhiqiang Luo
Summary: Researchers have successfully achieved direct electrical stimulation of peripheral nerves using a soft piezoelectric thin film nanogenerator remotely driven by programmable ultrasound pulses. This type of nanogenerator, without the need for additional rectifiers, can serve as a neurostimulator directly and the electrical pulses generated can be programmed by adjusting input power and waveform through ultrasound excitation.
Article
Genetics & Heredity
Yun Hao, Xiao-Lin Wang, Jun Xiao, Chun-Lei Jiao, Xin-Yao Meng, Jia-Chao Guo, Jing-Fan Shao, Jie-Xiong Feng, Jin-Peng He
Summary: The study investigated a family pedigree with craniometadiaphyseal dysplasia, revealing characteristic features related to common osteoporosis. Analysis showed distinct radiographic and clinical characteristics in the patient, with gene mutations transmitted from parents.
FRONTIERS IN GENETICS
(2021)
Review
Endocrinology & Metabolism
Yun Hao, Jia-Chao Guo, Xiao-Lin Wang, Jing-Fan Shao, Jie-Xiong Feng, Jin-Peng He
Summary: This study presented a group of children with a rare clinodactyly deformity caused by phalangeal intra-articular osteochondroma and evaluated the efficacy of various treatment methods. A retrospective analysis of 405 patients was conducted, with 8 cases included in the final analysis. Surgery was found to be the first choice of treatment for symptomatic osteochondromas, with successful outcomes and no recurrence recorded.
FRONTIERS IN ENDOCRINOLOGY
(2021)
Article
Oncology
Chenzhao Feng, Tianyu Xiang, Zixuan Yi, Xinyao Meng, Xufeng Chu, Guiyang Huang, Xiang Zhao, Feng Chen, Bo Xiong, Jiexiong Feng
Summary: This study applied deep learning model with attention mechanism to predict survivals in neuroblastoma with high accuracy. Two feature groups were found to be associated with patient prognosis and exhibited diverse molecular backgrounds.
FRONTIERS IN ONCOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Jing Zheng, Fei Long, Xu Cao, Bo Xiong, Yu Li
Summary: KATNAL2 mutations are associated with ASD and other related NDDs. In this study, katnal2 null allele was generated in zebrafish using CRISPR/Cas9 technology, and the resulting zebrafish showed developmental and behavioral defects, providing an in vivo model for studying the mechanisms underlying ASD related to KATNAL2 mutations.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Behavioral Sciences
Fei Long, Jing Zheng, Jiayi Zhou, Ping Hu, Bo Xiong
Summary: Tanc2 is a brain-expressed protein associated with autism spectrum disorder (ASD) and other neurodevelopmental disorders. Using a zebrafish model, researchers found that loss of tanc2 leads to an increase in brain size and body length, as well as an imbalance in excitatory and inhibitory neurons. These changes contribute to increased sleep, decreased anxiety-like behavior, reduced aggression, and impaired social preference.
