ArticleHepatology (Baltimore, Md.)2013
Placental stem cell correction of murine intermediate maple syrup urine disease.
Article in Hepatology (Baltimore, Md.), 2013. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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Who cites it
31 citing papers in PubMed, 67 citations in OpenAlex.
- Current Status of Cellular and Gene-Based Therapies for Congenital Metabolic Disorders: A Review.Medical science monitor : international medical journal of experimental and clinical research · 2026Review
- Coagulation activation and hepatic engraftment of human amnion-derived cells after intraportal transplantation in rats.Regenerative medicine · 2025Article
- Optimization of methods for intrasplenic administration of human amniotic epithelial cells in order to perform safe and effective cell-based therapy for liver diseases.Stem cell reviews and reports · 2024Article
- The biological functions and metabolic pathways of valine in swine.Journal of animal science and biotechnology · 2023Review
- Therapeutic Cell Repopulation of the Liver: From Fetal Rat Cells to Synthetic Human Tissues.Cells · 2023Review
- Inhibition of Epithelial-Mesenchymal Transition Maintains Stemness in Human Amniotic Epithelial Cells.Stem cell reviews and reports · 2022Article
- Review
- Human amniotic epithelial cell transplantation improves scar remodeling in a rabbit model of acute vocal fold injury: a pilot study.Stem cell research & therapy · 2022Article
- General consensus on multimodal functions and validation analysis of perinatal derivatives for regenerative medicine applications.Frontiers in bioengineering and biotechnology · 2022Review
- Amnion epithelial cells are an effective source of factor H and prevent kidney complement deposition in factor H-deficient mice.Stem cell research & therapy · 2021Article
- Clinical perspective on the use of human amniotic epithelial cells to treat congenital metabolic diseases with a focus on maple syrup urine disease.Stem cells translational medicine · 2021Article
- Characteristics and Therapeutic Potential of Human Amnion-Derived Stem Cells.International journal of molecular sciences · 2021Review
- Human Amniotic Epithelial Stem Cells: A Promising Seed Cell for Clinical Applications.International journal of molecular sciences · 2020Review
- Human Amnion Epithelial Cells Impair T Cell Proliferation: The Role of HLA-G and HLA-E Molecules.Cells · 2020Article
- Article
- Transcriptomic and computational analysis identified LPA metabolism, KLHL14 and KCNE3 as novel regulators of Epithelial-Mesenchymal Transition.Scientific reports · 2020Article
- Review
- Role of Toll-Like Receptors in Actuating Stem/Progenitor Cell Repair Mechanisms: Different Functions in Different Cells.Stem cells international · 2019Review
- The Immunomodulatory Properties of Amniotic Cells: The Two Sides of the Coin.Cell transplantation · 2018Article
- Evidence of Amniotic Epithelial Cell Differentiation toward Hepatic Sinusoidal Endothelial Cells.Cell transplantation · 2018Article
Corrections and comments
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Authors and funding
8 authors at 3 institutions in 2 countries.
Funding
Abstract
unlabelledThere is improved survival and partial metabolic correction of a mouse intermediate maple syrup urine disease (iMSUD) model after allogenic hepatocyte transplantation, confirming that a small number of enzyme-proficient liver-engrafted cells can improve phenotype. However, clinical shortages of suitable livers for hepatocyte isolation indicate a need for alternative cell sources. Human amnion epithelial cells (hAECs) share stem cell characteristics without the latter's safety and ethical concerns and differentiate to hepatocyte-like cells. Eight direct hepatic hAEC transplantations were performed in iMSUD mice over the first 35 days beginning at birth; animals were provided a normal protein diet and sacrificed at 35 and 100 days. Treatment at the neonatal stage is clinically relevant for MSUD and may offer a donor cell engraftment advantage. Survival was significantly extended and body weight was normalized in iMSUD mice receiving hAEC transplantations compared with untreated iMSUD mice, which were severely cachectic and died ≤28 days after birth. Branched chain α-keto acid dehydrogenase enzyme activity was significantly increased in transplanted livers. The branched chain amino acids leucine, isoleucine, valine, and alloisoleucine were significantly improved in serum and brain, as were other large neutral amino acids.
conclusionPlacental-derived stem cell transplantation lengthened survival and corrected many amino acid imbalances in a mouse model of iMSUD. This highlights the potential for their use as a viable alternative clinical therapy for MSUD and other liver-based metabolic diseases.
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