Publications by authors named "Xianglian Tang"

Clinically patients with thrombocytopenia are in urgent need of platelet transfusion, thus it is necessary to produce the platelets in large scale in vitro to meet the clinical needs. In this study, we developed efficient protocol to generate functioning platelets by differentiating umbilical cord blood (CB)-derived CD34 cells into mature megakaryocytes. Under our condition, up to 85% of mature megakaryocytes were generated from CB-derived CD34 cells, and over 75% CD42bCD62p platelets were produced.

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  • Autosomal recessive intellectual developmental disorder-3 is linked to mutations in a specific gene, resulting in intellectual disability and autism spectrum disorder, with limited genetic variants identified so far in affected patients.
  • A case study describes a Chinese patient with severe intellectual disability and autism, where whole-exome sequencing revealed a significant mutation, along with the discovery of six new likely pathogenic variants.
  • The research enhances understanding of the genetic and clinical features of this disorder, aiming to improve awareness, genetic testing, and counseling for affected families.
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  • Mega-corpus-callosum syndrome is a rare neurological disorder characterized by brain malformations, caused by mutations in the MAST1 gene, essential for neuron development.
  • A 26-year-old woman was referred for prenatal assessment after ultrasound showed significant brain abnormalities in her fetus, including enlarged ventricles and underdeveloped cerebellum.
  • Whole-exome sequencing revealed a specific mutation in the MAST1 gene, marking the first reported prenatal case of this disorder in the Chinese population and contributing to better understanding of the gene's mutation spectrum.
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  • The study investigates the genetic basis of nephronophthisis-related ciliopathies in a 3-year-old boy, identifying two significant mutations in a specific gene linked to multiple disorders including NPHP13 and Caroli disease.
  • Researchers performed whole-exome sequencing to analyze the boy's genetic variations, which included one novel splice-donor variant and a recurrent missense variant, as well as compound heterozygous variants.
  • The findings broaden the understanding of the genetic mutations associated with these disorders and emphasize the clinical diversity, aiding in genetic counseling and screening efforts within affected populations.
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Kabuki syndrome (KS) is a multiple congenital anomaly syndrome that is characterized by postnatal growth deficiency, hypotonia, short stature, mild-to-moderate intellectual disability, skeletal abnormalities, persistence of fetal fingertip pads, and distinct facial appearance. It is mainly caused by pathogenic/likely pathogenic variants in the or genes. Here, we described the clinical features of nine sporadic KS patients with considerable phenotypic heterogeneity.

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  • Human pluripotent stem cells (hPSCs) are crucial for regenerative medicine, but producing them at scale in 3D suspension cultures has been a challenge; dextran sulfate (DS) has shown promise in preventing excessive cell adhesion and large aggregate formation.
  • The study investigated the effects of DS on cellular adhesion molecules (CAMs) in hPSCs, revealing that DS treatment significantly down-regulated E-cadherin and intercellular adhesion molecule 1 (ICAM1), which are key players in hPSC adhesion.
  • Additionally, the research indicated that DS not only inhibited hPSC aggregation but also activated Wnt signaling pathways by up-regulating related genes, thereby influencing the expression of crucial CAMs involved in
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Background: Neuroblastoma (NB) is an infrequent childhood malignancy of the peripheral sympathetic nervous system and is accountable for about 10% of pediatric tumors. microRNA (miR)-125a has been implicated to serve as a tumor suppressor in various cancers. Herein, we set out to ascertain whether miR-125a exerts antitumor effects in NB.

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Background: Neuroblastoma (NB) comprises about 8-10% of pediatric cancers, and microRNA (miR)-338 downregulation has been implicated in NB. However, the underlying molecular mechanism remains largely unclear. The main goal of this study is to probe the regulatory role of miR-338 and the upstream and downstream biomolecules involved in NB.

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Integrin β1 (ITGB1), which acts as an extracellular matrix (ECM) receptor, has gained increasing attention as a therapeutic target for the treatment of hepatocellular carcinoma (HCC). However, the underpinning mechanism of how ITGB1 drives HCC progression remains elusive. In this study, we first found that ITGB1 expression was significantly higher in HCC tissues than in normal controls by bioinformatics analysis.

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Background: Exosomes secreted from stem cells exerted salutary effects on the fibrotic liver. Herein, the roles of exosomes derived from human embryonic stem cell (hESC) in anti-fibrosis were extensively investigated. Compared with two-dimensional (2D) culture, the clinical and biological relevance of three-dimensional (3D) cell spheroids were greater because of their higher regeneration potential since they behave more like cells in vivo.

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Objectives: For clinical applications of cell-based therapies, a large quantity of human pluripotent stem cells (hPSCs) produced in standardized and scalable culture processes is required. Currently, microcarrier-free suspension culture shows potential for large-scale expansion of hPSCs; however, hPSCs tend to aggregate during culturing leading to a negative effect on cell yield. To overcome this problem, we developed a novel protocol to effectively control the sizes of cell aggregates and enhance the cell proliferation during the expansion of hPSCs in suspension.

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Hepatocellular carcinoma (HCC), as a well-vascularized tumor, has attracted increasing attention in antiangiogenic therapies. Notably, emerging studies reveal that the long-term administration of antiangiogenic drugs induces hypoxia in tumors. Pericytes, which play a vital role in vascular stabilization and maturation, have been documented to be associated with antiangiogenic drug-induced tumor hypoxia.

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Rationale: Appendiceal intussusception is a rare disease. The definite preoperative diagnosis of appendiceal intussusception is rare and challenging. Here, we present a case of McSwain type V appendiceal intussusception in a 10-year-old boy.

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Long noncoding RNAs (lncRNAs) are a group of non-protein-coding transcripts exceeding 200 nucleotides in length, which are emerging as key players in various fundamental biological processes. Furthermore, it is increasingly recognized that mutation and dysregulation of lncRNAs contribute importantly to a variety of human diseases, particularly human cancers. Previous studies have revealed that altered lncRNAs have a close association with tumorigenesis, metastasis, prognosis and diagnosis of cancers.

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Rationale: Melanotic neuroectodermal tumor of infancy (MNTI) is an extremely rare benign pigmented neoplasm of neural crest origin with rapid expansile growth and a high recurrence rate. It is predominantly found in infants of <1 year of age, involvement of the head-and-neck region is the most common presentation though it is reported at other sites including mediastinum, shoulder, thigh, foot, epididymis, uterus and ovary. The patient reported here is the third case of MNTI presenting in an ovary, and the first reported in the infant ovary.

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