Papers

Importance of Monocarboxylate transporter 8 (Mct8) for the Blood-Brain Barrier Dependent Availability of 3,5,3’-Triiodo-L-Thyronine (T3)

Ainhoa Ceballos*, Monica M. Belinchon*, Eduardo Sanchez-Mendoza, Carmen Grijota- Martinez, Alexandra M. Dumitrescu, Samuel Refetoff, Beatriz Morte, and Juan Bernal Abstract:  Mutations of the gene expressing plasma membrane transporter for thyroid hormones MCT8 (SLC16A2) in humans lead to altered thyroid hormone levels and a severe neurodevelopmental disorder. Genetically engineered defect of the Mct8 gene in […]

Effects of thyroidectomy, T4, and DITPA replacement on brain blood vessel density in adult rats

Evelyn Heymann Schlenker,1 Megan Hora,1 Yingheng Liu,2 Rebecca A. Redetzke,2 Eugene Morkin,3 and A. Martin Gerdes2 Abstract:  Schlenker EH, Hora M, Liu Y, Redetzke RA, Morkin E, Gerdes AM. Effects of thyroidectomy, T4, and DITPA replacementon brain blood vessel density in adult rats. Am J Physiol Regul Integr Comp Physiol 294: R1504–R1509, 2008. First published […]

Novel Pathogenic Mechanism Suggested by Ex Vivo Analysis of MCT8 (SLC16A2) Mutations

W. Edward Visser,1 Jurgen Jansen,1 Edith C.H. Friesema,1 Monique H.A. Kester,1 Edna Mancilla,2 Johan Lundgren,3 Marjo S. van der Knaap,4 Roelineke J. Lunsing,5 Oebele F. Brouwer,5 and Theo J. Visser1 Abstract:  Monocarboxylate transporter 8 (MCT8; approved symbol SLC16A2) facilitates cellular uptake and efflux of 3,30,5-triiodothyronine (T3). Mutations in MCT8 are associated with severe psychomotor retardation, […]

Thyroid hormone transport in and out of cells

W. Edward Visser, Edith C.H. Friesema, Jurgen Jansen and Theo J. Visser Abstract:  Thyroid hormone (TH) is essential for the proper development of numerous tissues, notably the brain. TH acts mostly intracellularly, which requires transport by TH transporters across the plasma membrane. Although several transporter families have been identified, only monocarboxylate transporter (MCT)8, MCT10 and […]

Beneficial Effects of Propylthiouracil plus L-Thyroxine Treatment in a Patient with a Mutation in MCT8

J. L. We´ meau, M. Pigeyre, E. Proust-Lemoine, M. d’Herbomez, F. Gottrand, J. Jansen, T. J. Visser, and M. Ladsous Abstract:  Context: Mutations of the monocarboxylate transporter 8 (MCT8) gene determine a distinct Xlinked phenotype of severe psychomotor retardation and consistently elevated T3 levels. Lack of MCT8 transport of T3 in neurons could explain the […]

Pelizaeus–Merzbacher–Like Disease Presentation of MCT8 Mutated Male Subjects

Catherine Vaurs-Barrie`re, PhD,Marle`ne Deville, BS, Catherine Sarret, MD, Genevie`ve Giraud, BS, Vincent Des Portes, MD, PhD, Jose´-Maria Prats-Vin˜as, MD, PhD, Giuseppe De Michele, MD, Bernard Dan, MD, PhD, Angela F. Brady, MD, Odile Boespflug-Tanguy, MD, PhD, and Renaud Touraine, MD, PhD3 Abstract:  Pelizaeus–Merzbacher Disease is an X-linked hypomyelinatiing leukodystrophy. We report mutations in the thyroid […]

A Thyroid Hormone Analog with Reduced Dependence on the Monocarboxylate Transporter 8 for Tissue Transport

Caterina Di Cosmo, Xiao-Hui Liao, Alexandra M. Dumitrescu, Roy E. Weiss and Samuel Refetoff Abstract:  Mutations of the thyroid hormone (TH) cell membrane transporter MCT8, on chromosome-X, produce severe mental and neurological impairment in men. We generated a Mct8-deficient mouse (Mct8KO) manifesting the human thyroid phenotype. Although these mice have no neurological manifestations, they have […]

A Thyroid Hormone Analog with Reduced Dependence on the Monocarboxylate Transporter 8 for Tissue Transport

Caterina Di Cosmo, Xiao-Hui Liao, Alexandra M. Dumitrescu, Roy E. Weiss and Samuel Refetoff Abstract:  Mutations of the thyroid hormone (TH) cell membrane transporter MCT8, on chromosome-X, produce severe mental and neurological impairment in men. We generated a Mct8-deficient mouse (Mct8KO) manifesting the human thyroid phenotype. Although these mice have no neurological manifestations, they have […]