Publications by authors named "Penn A"

Neural activity is critical for sculpting the intricate circuits of the nervous system from initially imprecise neuronal connections. Disrupting the formation of these precise circuits may underlie many common neurodevelopmental disorders, ranging from subtle learning disorders to pervasive developmental delay. The necessity for sensory-driven activity has been widely recognized as crucial for infant brain development.

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In myasthenia gravis (MG) the muscle acetylcholine receptor (AChR) is the target of an autoimmune response. The anti-AChR response may originate in the thymus, which is abnormal in most MG patients and contains anti-AChR T and B cells. Microbial superantigens (sAg) may trigger autoimmune responses and in this study we sought clues as to whether sAg play a role in the pathogenesis of MG.

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This paper describes the diffusion of an evidence-based stroke guidance system (SGS) in a field setting through participatory research. SGS enables physicians to review relevant evidence-based literature, from which patient orders are generated for managing cerebrovascular accident. The paper focuses on the question 'what are the barriers and enablers to adopting SOS?' The research site consisted of eight hospitals within two health regions in Alberta, with 47 physicians as the intended users.

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When contacts are first forming in the developing nervous system, many neurons generate spontaneous activity that has been hypothesized to shape appropriately patterned connections. In Mustela putorius furo, monocular intraocular blockade of spontaneous retinal waves of action potentials by cholinergic agents altered the subsequent eye-specific lamination pattern of the lateral geniculate nucleus (LGN). The projection from the active retina was greatly expanded into territory normally belonging to the other eye, and the projection from the inactive retina was substantially reduced.

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Fractal dimension (fd) is a feature which is widely used to characterize medical images. Previously, researchers have shown that fd separates important classes of images and provides distinctive information about texture. We analyze limitations of two principal methods of estimating fd: box-counting (BC) and power spectrum (PS).

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Objectives: To investigate whether serum thiol levels are altered by HIV disease, and whether low serum thiols predict time to death among HIV-infected injecting drug users (IDU).

Design: A cross-sectional study of serum thiol levels among 13 HIV-seronegative IDU, 116 HIV-seropositive IDU, and 17 HIV-seropositive IDU with a history of AIDS, and a cohort study of the 133 HIV-infected IDU who took part in the cross-sectional study.

Methods: Subjects were recruited from a methadone-maintenance treatment program during 1990-1991.

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The nicotinic acetylcholine receptor (AChR) is a transmembrane glycoprotein composed of five homologous subunits. Different isoforms of the AChR alpha subunit exist (alpha 1 to alpha 9). Of them, alpha 1 is expressed in muscle, alpha 2 to alpha 9 in neuronal cells.

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Before vision, retinal ganglion cells produce spontaneous waves of action potentials. A crucial question is whether this spontaneous activity is transmitted to lateral geniculate nucleus (LGN) neurons. Using a novel in vitro preparation, we report that LGN neurons receive periodic barrages of postsynaptic currents from the retina that drive them to fire bursts of action potentials.

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1,3-Butadiene (BD), a gas widely used in the rubber industry, is also present in automotive exhaust and in the vapor phase of environmental tobacco smoke (ETS; approximately 400 micrograms/cigarette). The threshold limit value (TLV) for BD which was 10 ppm, has now been reduced to 2 ppm. Extensive investigations of workers have identified very few statistically significant increases in BD-associated cancer mortality.

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In addition to being the single greatest known environmental cause of cancer, cigarette smoke (CS) is also a major contributor to heart disease. We reported previously that 1) inhalation of either mainstream or sidestream CS promotes aortic arteriosclerotic plaque development; 2) 1,3 butadiene, a vapor-phase component of CS, promotes plaque development at 20 ppm, which at the time was only 2 times higher than the threshold limit value; and 3) individual tar fraction carcinogens in CS, including polynuclear aromatic hydrocarbons (PAHs) and nitrosamines, either do not promote plaque development or do so only at high concentrations. These results suggested that the tar fraction is not the primary source of plaque-promoting agents in CS.

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Kearns-Sayre syndrome is the most commonly diagnosed mitochondrial cytopathy and produces severe neuromuscular symptoms. The most frequent cause is a mitochondrial DNA deletion that removes a 4977-base pair segment of DNA that includes several genes encoding for respiratory chain subunits. Treatment of AIDS patients with nucleoside analogs has been reported to cause mtDNA depletion and myopathies.

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Article Synopsis
  • - A patient with severe COX deficiency and recurrent myoglobinuria has a 15-bp microdeletion in a key mitochondrial gene (COX subunit III).
  • - The mutant mitochondrial DNA (mtDNA) was predominantly found in muscle (92%) compared to a minimal amount in leukocytes (0.7%).
  • - Analysis of muscle fibers showed that COX-negative fibers had significantly more mutant mtDNA, marking the first molecular definition of isolated COX deficiency.
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Background: Our recent results support predictions from epidemiology studies that thousands of excess heart disease-related deaths result yearly in the United States from involuntary exposure to environmental tobacco smoke (ETS). Limited exposures of cockerels to ETS significantly accelerate arteriosclerosis. Despite little direct in vivo support, tar fraction rather than vapor phase compounds are considered largely responsible for the plaque-promoting effects of cigarette smoke.

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Objective: To explore mitochondrial dysfunction in Parkinson's disease (PD) using 31P magnetic resonance spectroscopy of resting muscle.

Design: Case-control study (28 PD patients and 28 normal controls) determining resting forearm inorganic phosphate/phosphocreatine (Pi/PCr) ratio.

Results: Significant difference (p = 0.

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A case is reported of severe unilateral hemispheric edema and localized hemorrhage associated with seizures following endarterectomy of an ipsilateral high-grade carotid stenosis. Imaging studies including angiography, computerized tomography (CT), magnetic resonance imaging/angiography, and xenon-CT, suggested postoperative ipsilateral cerebral hyperperfusion. Cerebral hyperperfusion syndromes caused by a probable failure of vascular autoregulation are rare but potentially serious complications after endarterectomy.

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A 170 kD protein, prominent in soluble extracts of rooster arteriosclerotic plaques, has been partially characterized. The protein was eluted from a size exclusion column in a broad molecular weight fraction > 100 kD. Concanavalin A and a murine polyclonal antibody raised against the isolated 170 kD protein reacted with the protein on Western blots.

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Involuntary exposure to environmental tobacco smoke (ETS) in public or in working places is considered to be a serious risk to human health. This symposium addressed several issues of toxicological interest that are associated with exposure to ETS. Epidemiologic evidence obtained in human studies suggests that "passive smoking" increases the risk of developing lung cancer in nonsmokers and favors the development of respiratory tract infections in children.

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Background: A number of epidemiologic studies have suggested that every year environmental tobacco smoke (second-hand smoke) is responsible for tens of thousands of deaths, mostly from heart disease, in the United States. Environmental tobacco smoke is composed mainly (80% to 85%) of aged and diluted sidestream smoke. The remainder is exhaled mainstream smoke.

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Kearns-Sayre syndrome (KSS) is a disease with severe clinical symptoms that often arises from a mitochondrial DNA deletion of 4977 bp. Quantification of defective mitochondrial DNA is important since the severity of symptoms in KSS is thought to be related to increased content of abnormal mitochondrial DNA. We developed a rapid, quantitative and competitive PCR assay to measure both wild-type and mutant forms of mitochondrial DNA in cells from KSS patients.

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During the first 3 weeks of postnatal development in the ferret retina, cells in the ganglion cell layer spontaneously generate waves of electrical activity that travel across the retina in the absence of mature photoreceptors (Meister et al., 1991; Wong et al., 1993).

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Andersen's syndrome is a clinically distinct form of potassium-sensitive periodic paralysis associated with cardiac dysrhythmias. The subtle nature of the cardiac and dysmorphic features may delay the recognition of this syndrome and its potentially lethal cardiac dysrhythmias. The genetic defect in Andersen's syndrome is not genetically linked to other forms of potassium-sensitive periodic paralysis and is probably distinct from the long QT syndrome locus.

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