Sciatic nerve analysis in thyroid hormone transporter Mct8 and Oatp1c1 knockout mice.

Objective: Mutations in the thyroid hormone (TH) transporter monocarboxylate transporter 8 (MCT8) cause Allan-Herndon-Dudley syndrome (AHDS), a severe form of psychomotor retardation with muscle hypoplasia and spastic paraplegia as key symptoms. These abnormalities have been attributed to an impaired TH transport across brain barriers and into neural cells thereby affecting brain development and function. Likewise, Mct8/Oatp1c1 (organic anion transporting polypeptide 1c1) double knockout (M/Odko) mice, a well-established murine AHDS model, display a strongly reduced TH passage into the brain as well as locomotor abnormalities.

Psychotropic medication usage in sporadic versus genetic behavioral-variant frontotemporal dementia.

Introduction: Psychotropic medication (PM) use in behavioral-variant frontotemporal dementia (bvFTD) is higher than in other dementias. However, no information exists on whether PM use differs between sporadic and genetic bvFTD.

Methods: We analyzed data from sporadic and genetic bvFTD participants with PM prescriptions in the Advancing Research and Treatment in Frontotemporal Lobar Degeneration/Longitudinal Evaluation of Familial Frontotemporal Dementia Subjects study.

The functional role of the task-irrelevant stimulus feature in the congruency sequence effect.

Humans have to deal with conflicting information. This is studied in conflict tasks such as the Simon task or the flanker task. For example, participants respond with the left or right hand to the color of a stimulus (task-relevant stimulus feature) which is presented in a left or right position (irrelevant feature) in the Simon task or to a letter (relevant) which is flanked by same or different letters (irrelevant) in the flanker task.

Where does the processing of size meet the processing of space?

Previous studies revealed an S-R compatibility effect between physical stimulus size and response location, with faster left (right) responses to small (large) stimuli, respectively, as compared to the reverse assignments. Here, we investigated the locus of interactions between the processing of size and spatial locations. In Experiment 1, we explored whether stimulus size and stimulus location interact at a perceptual level of processing when responses lack spatiality.

Toward a treatment for thyroid hormone transporter MCT8 deficiency - achievements and challenges.

Patients with an inactive thyroid hormone (TH) transporter MCT8 (Allan-Herndon-Dudley Syndrome, AHDS) display severe neurological impairments and motor disabilities, indicating an indispensable function of MCT8 in facilitating TH access to the human brain. Consequently, the CNS of AHDS patients appears to be in a TH deficient state, which greatly compromises proper neural development and function. Another hallmark of this disease is that patients exhibit elevated serum T3 levels, leading to a hyperthyroid situation in peripheral tissues.