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Batten disease, a degenerative neurological disorder with juvenile onset, is the most common form of the neuronal ceroid lipofuscinoses. Mutations in the CLN3 gene cause Batten disease. To facilitate studies of Batten disease pathogenesis and treatment, a murine model was created by targeted disruption of the Cln3 gene. Mice homozygous for the disrupted(More)
The OECD conventional 28-day repeat dose toxicity test (OECD TG 407) is widely employed in the initial hazard identification and characterization for commercial chemicals. The OECD has recently undertaken an international effort to “enhance” the conventional 28-day repeat dose toxicity test (OECD TG 407) in order to ensure that chemicals acting through(More)
1. The directional variation of kinematic and electromyographic (EMG) characteristics of two-joint arm movements made to targets in a two-dimensional work space was studied in monkeys trained to make targeted arm movements under different behavioral conditions. 2. In each animal, kinematic measures of movement (movement amplitude, movement time, peak(More)
Pancreatic exocrine function tests and plasma insulin curves after a glucose load have been performed in 74 patients, 32 with no significant pancreatic disease, 31 with various disorders of the pancreas, and 11 with postmature diabetes mellitus. Exocrine function was impaired in all patients with chronic pancreatitis and chronic relapsing pancreatitis,(More)
A current issue for regulatory agencies is endocrine-related modes of action such as those mediated by the estrogen, androgen, and thyroid nuclear receptors. At the national and international levels, the consensus recommendation for the assessment of such modes of action is a tiered series of in vitro and in vivo protocols. The tiered framework begins with(More)
OBJECTIVE Hepatitis C virus (HCV) infection is the leading cause of liver failure in adulthood. Although the prevalence of HCV is reportedly as high as 80% in incarcerated adult populations, little is known about the prevalence of HCV in incarcerated juvenile populations. The purpose of this study was to determine the prevalence of HCV and high-risk(More)
Childhood epilepsy can be severe and even catastrophic. In these instances, cognition can be impaired-leading to long-term intellectual disabilities. One factor that could potentially cause cognitive deficits is the frequent seizures that characterize intractable epilepsy. However, it has been difficult to separate the effects seizures may have from those(More)
The effects recurring seizures have on the developing brain are an important area of debate because many forms of human epilepsy arise in early life when the central nervous system is undergoing dramatic developmental changes. To examine effects on glutamatergic synaptogenesis, epileptiform activity was induced by chronic treatment with GABAa receptor(More)
The classification scheme for human health indicators in life-cycle assessment (LCA) and other toxicity scores is proposed based on identifiable target organ or system toxicity. This scheme represents a compromise between a subjective single overall score and highly detailed toxic mechanisms of action. A case study was used to evaluate the proposed(More)