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Synthetic Augmentation of Bilirubin Metabolism in Human Pluripotent Stem Cell-derived Liver Organoids

ABSTRACT: Synthetic Augmentation of Bilirubin Metabolism in Human Pluripotent Stem Cell-derived Liver Organoids

PROVIDER: PRJNA919091 | ENA |

REPOSITORIES: ENA

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Synthetic Augmentation of Bilirubin Metabolism in Human Pluripotent Stem Cell-derived Liver Organoids

Project description:To investigate the efect of perturbation of glucocorticoid signaling on the difference between the biliurbin metabolism in HLOs after treatment with drugs.

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AAV-mediated gene augmentation therapy of CRB1-patient derived retinal organoids restores the histological and transcriptional retinal phenotype

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Proteome analysis of brainstem organoids from human pluripotent stem cells

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Retinal organoids with X-linked retinoschisis RS1 (E72K) mutation exhibit a photoreceptor developmental delay and are rescued by gene augmentation therapy

Project description:Background X-linked juvenile retinoschisis (XLRS) is an inherited disease caused by RS1 gene mutation, which leads to retinal splitting and visual impairment. The mechanism of RS1-associated retinal degeneration is not fully understood. Besides, animal models of XLRS have limitations in the study of XLRS. Here, we used human induced pluripotent stem cell (hiPSC)-derived retinal organoids (ROs) to investigate the disease mechanisms and potential treatments for XLRS. Methods hiPSCs reprogrammed from peripheral blood mononuclear cells of two RS1 mutant (E72K) XLRS patients were differentiated into ROs. Subsequently, we explored whether RS1 mutation could affect RO development and explore the effectiveness of RS1 gene augmentation therapy. Results ROs derived from RS1 (E72K) mutation hiPSCs exhibited a developmental delay in the photoreceptor, retinoschisin (RS1) deficiency, and altered spontaneous activity compared with control ROs. Furthermore, the delays in development were associated with decreased expression of rod-specific precursor markers (NRL) and photoreceptor-specific markers (RCVRN). Adeno-associated virus (AAV)-mediated gene augmentation with RS1 at the photoreceptor immature stage rescued the rod photoreceptor developmental delay in ROs with the RS1 (E72K) mutation. Conclusions The RS1 (E72K) mutation results in the photoreceptor development delay in ROs and can be partially rescued by the RS1 gene augmentation therapy.

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Combination of a microfluidic device with brain extracellular matrix promotes structural and functional maturation of human brain organoids

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