A novel, ataxic mouse model of ataxia telangiectasia caused by a clinically relevant nonsense mutation.

Ataxia Telangiectasia (A-T) and Ataxia with Ocular Apraxia Type 1 (AOA1) are devastating neurological disorders caused by null mutations in the genome stability genes, A-T mutated () and Aprataxin (), respectively. Our mechanistic understanding and therapeutic repertoire for treating these disorders are severely lacking, in large part due to the failure of prior animal models with similar null mutations to recapitulate the characteristic loss of motor coordination (i.e.

Limitations to vasodilatory capacity and .VO2 max in trained human skeletal muscle.

To further explore the limitations to maximal O(2) consumption (.VO(2 max)) in exercise-trained skeletal muscle, six cyclists performed graded knee-extensor exercise to maximum work rate (WR(max)) in hypoxia (12% O(2)), hyperoxia (100% O(2)), and hyperoxia + femoral arterial infusion of adenosine (ADO) at 80% WR(max). Arterial and venous blood sampling and thermodilution blood flow measurements allowed the determination of muscle O(2) delivery and O(2) consumption.

Smoking in contemporary American cinema.

Objective: The true prevalence of smoking among characters portrayed in the movies is unknown. This study examines this prevalence objectively.

Design: The top 10 movies on the weekly box office charts were reviewed.

Continuous transforming growth factor beta1 secretion by cell-mediated gene therapy maintains chondrocyte redifferentiation.

One of the most important factors in the production of cartilage is transforming growth factor beta1 (TGF-beta1). To obtain sustained release of TGF-beta1, a cell-mediated gene therapy technique was introduced. We infected chondrocytes with a retroviral vector carrying the TGF-beta1 gene.

Anaphylactoid response to Optison(R) and its effects on pulmonary function in two dogs.

Two of 26 anesthetized dogs given the cardiac echo-enhancing agent Optison showed anaphylactoid responses (AR) related to the human albumin component of this agent. The episodes of AR were self-limited, and could be reproduced by human albumin injection alone. Gas exchange was maintained by mechanical ventilation and 5 cm H(2)O PEEP, and dispersion of ventilation remained normal during AR despite severe hypotension.

Regulation of free radical outflow from an isolated muscle bed in exercising humans.

Incremental knee extensor (KE) exercise performed at 25, 70, and 100% of single-leg maximal work rate (WR(MAX)) was combined with ex vivo electron paramagnetic resonance (EPR) spectroscopic detection of alpha-phenyl-tert-butylnitrone (PBN) adducts, lipid hydroperoxides (LH), and associated parameters in five males. Blood samples were taken from the femoral arterial and venous circulation that, when combined with measured changes in femoral venous blood flow, permitted a direct examination of oxidant exchange across a functionally isolated contracting muscle bed. KE exercise progressively increased the net outflow of LH and PBN adducts (100% > 70% > 25% WR(MAX), P < 0.

Effects of Optison on pulmonary gas exchange and hemodynamics.

Optison is a contrast-enhancing agent used in myocardial contrast echocardiography. It consists of small albumin spherules (approximately 4-microm diameter) containing a fluorocarbon gas, octafluoropropane. It is injected IV and, thus, may cause pulmonary manifestations of microembolism.