Dissociating executive function and ADHD influences on reading ability in children with dyslexia.

Developmental dyslexia (DD) and attention-deficit/hyperactivity disorder (ADHD) are two of the most common neurodevelopmental disorders among school-age children. These disorders frequently co-occur, with up to 40-50% of children with one diagnosis meeting criteria for the other, and similar percentages of children with either DD or ADHD exhibiting impaired executive functions (EF). Although both ADHD and EF deficits are common in dyslexia, there is little evidence about how ADHD and EF deficits specifically influence the brain basis of reading difficulty in dyslexia, and whether the influences of ADHD and EF on dyslexia can be disentangled.

Nervous system involvement in von Hippel-Lindau disease: pathology and mechanisms.

Patients with von Hippel-Lindau disease carry a germline mutation of the Von Hippel-Lindau (VHL) tumor-suppressor gene. We discuss the molecular consequences of loss of VHL gene function and their impact on the nervous system. Dysfunction of the VHL protein causes accumulation and activation of hypoxia inducible factor (HIF) which can be demonstrated in earliest stages of tumorigenesis and is followed by expression of VEGF, erythropoietin, nitric oxide synthase and glucose transporter 1 in VHL-deficient tumor cells.

Developmentally arrested structures preceding cerebellar tumors in von Hippel-Lindau disease.

There is increasing evidence that suggests that knockout of tumor-suppressor gene function causes developmental arrest and protraction of cellular differentiation. In the peripheral nervous system of patients with the tumor-suppressor gene disorder, von Hippel-Lindau disease, we have demonstrated developmentally arrested structural elements composed of hemangioblast progenitor cells. Some developmentally arrested structural elements progress to a frank tumor, hemangioblastoma.

Hypothermia and ERK activation after cardiac arrest.

Mild hypothermia improves survival and neurological outcome after cardiac arrest, as well as increasing activation of the extracellular-signal-regulated kinase (ERK) in hippocampus. ERK signaling is involved in neuronal growth and survival. We tested the hypothesis that the beneficial effects of hypothermia required ERK activation.

Small molecule activators of the heat shock response and neuroprotection from stroke.

Various cellular defense pathways are mobilized in response to stress that serve to limit potential damage to organelles and biochemical pathways that would disrupt normal cellular function or trigger cell death. Strategies utilized by cells subjected to various forms of stress include the activation of detoxification systems that act to eliminate the primary damaging molecules, remove damaged cellular macromolecules, or restore organelle and macromolecule function in cases where loss of activity is generated by reversible modifications or alterations in conformation (ie, misfolding). Central to many intracellular defense mechanisms that operate to limit damage to protein function are molecular chaperones of the heat shock protein (HSP) family.

Subicular dendritic arborization in Alzheimer's disease correlates with neurofibrillary tangle density.

Intracellular accumulation of PHFtau in Alzheimer's disease (AD) disrupts the neuronal cytoskeleton and other neuronal machinery and contributes to axonal and dendritic degeneration, and neuronal death. Furthermore, amyloid-beta (Abeta) has been reported to be toxic to neurons and neurites. While loss of presynaptic elements is an established feature of AD, the nature and extent of dendritic degeneration has been infrequently studied.