Late diagnosis of Marfan syndrome with fatal outcome in a young male patient: a case report.

Introduction: Marfan syndrome is a heritable disorder of the connective tissue that affects many organ systems. However, the most serious complication in patients with Marfan syndrome is progressive aortic root dilation, which may lead to aortic dissection, rupture or aortic regurgitation. Prevention of these life threatening complications is of major importance.

A study of coupling interactions in finite arbitrarily-shaped grooves in electromagnetic scattering problem.

A study of coupling between finite number of general-shaped grooves in electromagnetic plane-wave scattering problem is presented. The formulation for a single groove [1] is extended to two grooves. The importance of inclusion of coupling interaction between two grooves in scattering analysis is presented and its dependence on the grooves separation distance and the angle of incident of the electromagnetic field is demonstrated quantitatively.

Modulating effect of Withania somnifera on TCA cycle enzymes and electron transport chain in azoxymethane-induced colon cancer in mice.

The aim of the present investigation was to evaluate the efficacy of Withania somnifera on tricarboxylic acid (TCA) cycle enzymes and electron transport chain in azoxymethane-induced experimental colon cancer in mice. Azoxymethane at the dose of 15 mg/kg body weight was induced intraperitoneally once in a week for 28 days. The progression in colon tumor development was correlated with the appearance of the histological biomarker and aberrant crypt foci (ACF).

Tolfenamic acid decreases c-Met expression through Sp proteins degradation and inhibits lung cancer cells growth and tumor formation in orthotopic mice.

The nonsteroidal anti-inflammatory drug (NSAID), tolfenamic acid (TA) is emerging as a new anti-cancer agent. TA induces the degradation of specific Specificity protein (Sp) transcription factors, Sp1, Sp3 and Sp4 which are associated with tumor growth and metastasis. In this study we have evaluated the effect of TA on lung cancer using both in vitro and in vivo models.

Functional significance of muscarinic receptor expression within the proximal and distal rat vagina.

Information regarding the role of cholinergic nerves in mediating vaginal smooth muscle contraction is sparse, and in vitro studies of the effects of muscarinic agonists on vaginal smooth muscle are discrepant. The goal of this study was to determine the expression of muscarinic receptors in the vaginal wall of the rat. In addition, we sought to determine the effect of the muscarinic receptor agonist carbachol on contractility and inositol phosphate production of the proximal and distal rat vaginal muscularis.

Rare sensory and autonomic disturbances associated with vitamin B12 deficiency.

Vitamin B12 deficiency is an important nutritional disorder causing neurological manifestations of myelopathy, neuropathy and dementia. Sub-acute combined degeneration (SCD) with involvement of the posterior columns in the cervical and thoracic cord is a common presentation of this disorder. In this case report, we describe a 43 year old woman with pernicious anemia and myelopathy with atypical clinical features.

Lifespan profiles of Alzheimer's disease-associated genes and products in monkeys and mice.

Alzheimer's disease (AD) is characterized by plaques of amyloid-beta (Abeta) peptide, cleaved from amyloid-beta protein precursor (AbetaPP). Our hypothesis is that lifespan profiles of AD-associated mRNA and protein levels in monkeys would differ from mice and that differential lifespan expression profiles would be useful to understand human AD pathogenesis. We compared profiles of AbetaPP mRNA, AbetaPP protein, and Abeta levels in rodents and primates.

Tolfenamic acid enhances pancreatic cancer cell and tumor response to radiation therapy by inhibiting survivin protein expression.

Survivin is overexpressed in most human cancers, including pancreatic adenocarcinoma. Expression of survivin is regulated by specificity protein (Sp) proteins and related to resistance to radiation therapy. Tolfenamic acid induces Sp protein degradation in several cancer cell lines.

Densitometric analysis of dental implant placement between flapless technique and the two-stage technique--a pilot study.

Flapless technique is a surgical approach of implant placement without raising a mucoperiosteal flap. Such approach has many advantages: shorter surgical treatment, minimal bleeding, postoperative discomfort for the patient is reduced; possibility of immediate loading of the inserted implant, faster procedure of implant placement and by that less time is needed for the complete implant-prosthetic restoration. Purpose of this pilot study was radiographic assessment of flapless technique and determination of its clinical values in comparison with two-stage dental implant technique through computerized densitometric analysis.

Alzheimer's disease (AD)-like pathology in aged monkeys after infantile exposure to environmental metal lead (Pb): evidence for a developmental origin and environmental link for AD.

The sporadic nature of Alzheimer's disease (AD) argues for an environmental link that may drive AD pathogenesis; however, the triggering factors and the period of their action are unknown. Recent studies in rodents have shown that exposure to lead (Pb) during brain development predetermined the expression and regulation of the amyloid precursor protein (APP) and its amyloidogenic beta-amyloid (Abeta) product in old age. Here, we report that the expression of AD-related genes [APP, BACE1 (beta-site APP cleaving enzyme 1)] as well as their transcriptional regulator (Sp1) were elevated in aged (23-year-old) monkeys exposed to Pb as infants.

The environment, epigenetics and amyloidogenesis.

Alzheimer's Disease (AD) is a progressive, irreversible neurodegenerative disease. Despite several genetic mutations (Haass et al., J.

New and evolving concepts in the neurotoxicology of lead.

Lead (Pb) is a xenobiotic metal with no known essential function in cellular growth, proliferation, or signaling. Decades of research characterizing the toxicology of Pb have shown it to be a potent neurotoxicant, especially during nervous system development. New concepts in the neurotoxicology of Pb include advances in understanding the mechanisms and cellular specificity of Pb.

Environmental and dietary risk factors in Alzheimer's disease.

Alzheimer's disease (AD) is a progressive neurodegenerative disease that affects millions in the aging population worldwide and will affect millions more in the next 20 years. Over 90% of all cases are sporadic, with genetics playing a minor role in the etiology of AD. Therefore, it is crucial to investigate the environment and diet as primary risk factors in AD pathology.

Rigorous formulation for electromagnetic plane-wave scattering from a general-shaped groove in a perfectly conducting plane.

Scattering of an obliquely incident plane wave by a general-shaped groove engraved on a perfectly conducting plane is rigorously solved. The scattered field is represented by a Fourier-integral representation. To analytically represent the fields in a general-shaped groove, the groove is divided into L number of layers.

How and when environmental agents and dietary factors affect the course of Alzheimer's disease: the "LEARn" model (latent early-life associated regulation) may explain the triggering of AD.

Alzheimer's disease (AD) is currently the most prominent form of dementia among the elderly. Although AD manifests in late adult life, it is not clear when the disease actually starts and how long the neuropathological processes take to develop AD. The major unresolved question is the timing and the nature of triggering leading to AD.

Lead exposure: expression and activity levels of Oct-2 in the developing rat brain.

Lead is a highly neurotoxic metal, and the developing central nervous system is particularly vulnerable to the effects of lead. In this study, transcription factors (TFs) that are altered due to lead exposure were identified using macroarray analysis. Rat pups were lactationally exposed to 0.

Regional differences in myosin heavy chain isoform expression and maximal shortening velocity of the rat vaginal wall smooth muscle.

Contractility of the proximal and distal vaginal wall smooth muscle may play distinct roles in the female sexual response and pelvic support. The goal of this study was to determine whether differences in contractile characteristics of smooth muscle from these regions reside in differences in the expression of isoforms of myosin, the molecular motor for muscle contraction. Adult female Sprague-Dawley rats were killed on the day of estrus, and the vagina was dissected into proximal and distal segments.

Environmental risk factors and the developmental basis for Alzheimer's disease.

Alzheimer's disease (AD) is a progressive neurodegenerative disorder whose clinical manifestations appear in old age. The hallmark pathological features of AD (amyloid plaques and associated proteins) are present in normal aging indivduals, suggesting that AD may result from the acceleration of normal age-related processes in the brain. The sporadic nature of most AD cases strongly argues for an environmental link that may drive AD pathogenesis; however, it is unclear when this environmental stress may occur.

Lead (Pb) exposure and its effect on APP proteolysis and Abeta aggregation.

Alzheimer's disease (AD) is a progressive neurodegenerative disorder with clinical manifestations appearing in old age, however, the initial stages of this disease may begin early in life. AD is characterized by the presence of excessive deposits of aggregated beta-amyloid (Abeta) peptides, which are derived from the beta-amyloid precursor protein (APP) following processing by beta-secretase and gamma-secretase. Recently, we have reported that developmental exposure of rats to Pb resulted in latent elevation of APP mRNA, APP, and Abeta in old age.

Ontogenetic alterations in prototypical transcription factors in the rat cerebellum and hippocampus following perinatal exposure to a commercial PCB mixture.

Polychlorinated biphenyls (PCBs) are prevalent in the environment despite the ban of their use for decades and offer a model system to understand developmental neurotoxicity of persistent pollutants. Disturbances in brain development and cognition are among the neurotoxic manifestations of PCBs. The cellular and molecular basis for PCB-induced developmental neurotoxicity is still unclear; however, a series of in vitro and some in vivo studies have revealed that the disruption of Ca(2+) homeostasis and Ca(2+)-mediated signal transduction play a significant role.

The fetal basis of amyloidogenesis: exposure to lead and latent overexpression of amyloid precursor protein and beta-amyloid in the aging brain.

The fetal basis of adult disease (FeBAD) hypothesis states that many adult diseases have a fetal origin. According to FeBAD, injury or environmental influences occurring at critical periods of organ development could result in "programmatic" changes via alterations in gene expression or gene imprinting that may result in functional deficits that become apparent later in life. Alzheimer's disease (AD) is a progressive neurodegenerative disorder that is characterized by excessive deposits of aggregated beta-amyloid (Abeta) peptides, which are snippets of the beta-amyloid precursor protein (APP).

Determining the ability of xenobiotic metals to bind a specific protein domain by electrophoresis.

Environmental metals are potentially toxic and can interfere with the metal-binding motifs of various critical proteins. This unit describes an electrophoretic method that can be used to measure the ability of a xenobiotic metal to bind a zinc-finger motif. Information gained using this protocol can lead to the identification of protein targets of metals and shed better light on their mechanisms of action.

Urinary bladder perforation in a premature infant with Down syndrome.

Urinary bladder perforation due to bladder catheterization in neonates is a rare iatrogenic complication. It has been reported secondary to various causes and a variety of surgical settings in neonates. A case of urinary bladder perforation due to catheterization in a premature baby with Down syndrome, who presented with progressive renal failure and mild-to-moderate ascites, is reported.

Lead induced effects on acetylcholinesterase activity in cerebellum and hippocampus of developing rat.

Exposure to low-levels of lead (Pb) during early development has been implicated in behavioral abnormalities and cognitive deficits in children. The present study is focused on developmental changes in hippocampus and cerebellum of rats following perinatal exposure to Pb. Pregnant rats were exposed to 0.

Intracellular signaling pathways involved in mediating the effects of lead on the transcription factor Sp1.

It has been well established that exposure to Pb during critical periods of brain development results in both cognitive and behavioral deficits. Although the mechanism by which Pb induces developmental neurotoxicity is unknown, it may involve alterations in transcription of genes that are essential for growth and differentiation. Recent studies reveal that Pb interferes with growth and differentiation by acting on the transcription factor Sp1.