دورية أكاديمية

Oligodendrocyte progenitor cell maturation is dependent on dual function of MCT8 in the transport of thyroid hormone across brain barriers and the plasma membrane.

التفاصيل البيبلوغرافية
العنوان: Oligodendrocyte progenitor cell maturation is dependent on dual function of MCT8 in the transport of thyroid hormone across brain barriers and the plasma membrane.
المؤلفون: Vatine GD; The Department of Physiology and Cell Biology, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.; The Regenerative Medicine and Stem Cell (RMSC) Research Center, Ben-Gurion University of the Negev, Beer Sheva, Israel.; The Zlotowski Center for Neuroscience, Ben-Gurion University of the Negev, Beer Sheva, Israel., Shelest O; The Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA., Barriga BK; The Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA., Ofan R; The Department of Physiology and Cell Biology, Faculty of Health Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel.; The Regenerative Medicine and Stem Cell (RMSC) Research Center, Ben-Gurion University of the Negev, Beer Sheva, Israel.; The Department of Biotechnology Engineering, Faculty of Engineering Sciences, Ben-Gurion University of the Negev, Beer Sheva, Israel., Rabinski T; The Regenerative Medicine and Stem Cell (RMSC) Research Center, Ben-Gurion University of the Negev, Beer Sheva, Israel., Mattis VB; The Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.; FUJIFILM Cellular Dynamics Inc., Madison, Wisconsin, USA., Heuer H; Department of Endocrinology, Diabetes and Metabolism, University of Duisburg-Essen, Essen, Germany., Svendsen CN; The Board of Governors Regenerative Medicine Institute, Cedars-Sinai Medical Center, Los Angeles, California, USA.
المصدر: Glia [Glia] 2021 Sep; Vol. 69 (9), pp. 2146-2159. Date of Electronic Publication: 2021 May 06.
نوع المنشور: Journal Article; Research Support, Non-U.S. Gov't
اللغة: English
بيانات الدورية: Publisher: Wiley-Liss Country of Publication: United States NLM ID: 8806785 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1098-1136 (Electronic) Linking ISSN: 08941491 NLM ISO Abbreviation: Glia Subsets: MEDLINE
أسماء مطبوعة: Publication: New York, NY : Wiley-Liss
Original Publication: New York : Alan R. Liss, Inc., c1988-
مواضيع طبية MeSH: Oligodendrocyte Precursor Cells*/metabolism , Symporters*/genetics , Symporters*/metabolism, Animals ; Brain/metabolism ; Cell Membrane/metabolism ; Humans ; Mice ; Monocarboxylic Acid Transporters/metabolism ; Thyroid Hormones/genetics ; Thyroid Hormones/metabolism
مستخلص: Inactivating mutations in the thyroid hormone (TH) transporter monocarboxylate transporter 8 (MCT8) causes a rare and debilitating form of X-linked psychomotor disability known as Allan Herndon Dudley syndrome (AHDS). One of the most prominent pathophysiological symptoms of MCT8-deficiency is hypomyelination. Here, patient-derived induced pluripotent stem cells (iPSCs) were used to study the role of MCT8 and TH on the maturation of oligodendrocytes. Interestingly, neither MCT8 mutations nor reduced TH affected the in vitro differentiation of control or MCT8-deficient iPSCs into oligodendrocytes. To assess whether patient-derived iPSC-derived oligodendrocyte progenitor cells (iOPCs) could provide myelinating oligodendrocytes in vivo, cells were transplanted into the shiverer mouse corpus callosum where they survived, migrated, and matured into myelinating oligodendrocytes, though the myelination efficiency was reduced compared with control cells. When MCT8-deficient and healthy control iOPCs were transplanted into a novel hypothyroid immunodeficient triple knockout mouse (tKO, mct8 -/- ; oatp1c1 -/- ; rag2 -/- ), they failed to provide behavioral recovery and did not mature into oligodendrocytes in the hypothyroid corpus callosum, demonstrating the critical role of TH transport across brain barriers in oligodendrocyte maturation. We conclude that MCT8 plays a cell autonomous role in oligodendrocyte maturation and that functional TH transport into the central nervous system will be required for developing an effective treatment for MCT8-deficient patients.
(© 2021 Wiley Periodicals LLC.)
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فهرسة مساهمة: Keywords: MCT8; OPCs; blood brain barrier; thyroid hormone; transplantation
المشرفين على المادة: 0 (Monocarboxylic Acid Transporters)
0 (Symporters)
0 (Thyroid Hormones)
تواريخ الأحداث: Date Created: 20210506 Date Completed: 20220310 Latest Revision: 20220311
رمز التحديث: 20231215
DOI: 10.1002/glia.24014
PMID: 33956384
قاعدة البيانات: MEDLINE
الوصف
تدمد:1098-1136
DOI:10.1002/glia.24014