James McKenna

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Tuberous sclerosis complex (TSC) is an autosomal dominant, tumor predisposition disorder characterized by significant neurodevelopmental brain lesions, such as tubers and subependymal nodules. The neuropathology of TSC is often associated with seizures and intellectual disability. To learn about the developmental perturbations that lead to these brain(More)
Tuberous sclerosis complex (TSC) is a dominant tumor suppressor disorder caused by mutations in either TSC1 or TSC2. TSC causes substantial neuropathology, often leading to autism spectrum disorders (ASDs) in up to 60% of patients. The anatomic and neurophysiologic links between these two disorders are not well understood. We have generated and(More)
Coastal waters are severely threatened by nitrogen (N) loading from direct groundwater discharge. The subterranean estuary, the mixing zone of fresh groundwater and sea water in a coastal aquifer, has a high potential to remove substantial N. A network of piezometers was used to characterize the denitrification capacity and groundwater flow paths in the(More)
In this paper a method for calculating the first and higher order moments of the queue lengths for each customer class at each node in a dosed Markovian queuing network is presented. The method is based on asymptotic expansions in powers of N -~ for the moments of interest. N is a parameter that reflects the size of the network. The derivation of the(More)
Tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by mutations in the TSC1 or TSC2 gene. Patients afflicted with TSC develop tumors in various organ systems, but cerebral pathology is particularly severe. Conventional gene disruption of the Tsc1 or Tsc2 gene in mice cause limited central nervous system pathology. Homozygous deletion(More)
Tuberous sclerosis complex (TSC) is a neurogenetic disorder that often causes brain abnormalities leading to epilepsy, developmental delay, and autism. TSC is caused by inactivating mutations in either of the genes encoding the proteins hamartin (TSC1) and tuberin (TSC2). These proteins form a heterodimer that inhibits the mammalian target of rapamycin(More)
Tuberous sclerosis complex (TSC) is caused by heterozygous mutations in either the TSC1 (hamartin) or the TSC2 (tuberin) gene. Among the multisystemic manifestations of TSC, the neurodevelopmental features cause the most morbidity and mortality, presenting a considerable clinical challenge. Hamartin and tuberin form a heterodimer that inhibits the mammalian(More)
The genetic disease tuberous sclerosis complex (TSC) is an autosomal dominant disorder caused by loss of function mutations in either TSC1 (hamartin) or TSC2 (tuberin), which serve as negative regulators of mechanistic target of rapamycin complex 1 (mTORC1) activity. TSC patients exhibit developmental brain abnormalities and tuber formations that are(More)
Striking progress has been made recently in obtaining expressions for the sojourn time distribution function (STDF) of a job at a <italic>c</italic>-server, first-come, first-serve (FCFS) center in a closed, product-form queuing network. These results have more recently been extended, and expressions have been obtained for the joint distribution function(More)