Anti-BAAT (aa 258-355) polyclonal antibody

Bile acid amidation defects were predicted to present with fat/fat soluble vitamin malabsorption with minimal cholestasis. We identified and treated five patients (one male, four females) from four families with defective bile acid amidation due to a genetically confirmed deficiency in bile acid CoA:amino acid N-acyl transferase (BAAT) with the conjugated bile acid, glycocholic acid (GCA). Fast atom bombardment-mass spectrometry analysis of urine and bile at baseline revealed predominantly unconjugated cholic acid and absence of the usual glycine and taurine conjugated primary bile acids. Treatment with 15 mg/kg GCA resulted in total duodenal bile acid concentrations of 23.3 +/- 19.1 mmol/L (mean +/- SD) and 63.5 +/- 4.0% of the bile acids were secreted in bile in the conjugated form, of which GCA represented 59.6 +/- 9.3% of the total biliary bile acids. Unconjugated cholic acid continued to be present in high concentrations in bile because of partial intestinal deconjugation of orally administered GCA. Serum total bile acid concentrations did not significantly differ between pretreatment and posttreatment samples and serum contained predominantly unconjugated cholic acid. These findings confirmed efficient intestinal absorption, hepatic extraction, and biliary secretion of the administered GCA. Oral tolerance tests for vitamin D-2 (1,000 IU vitamin D-2/kg) and tocopherol (100 IU/kg tocopherol acetate) demonstrated improvement in fat-soluble vitamin absorption after GCA treatment. Growth improved in 3/3 growth-delayed prepubertal patients. Conclusion: Oral glycocholic acid therapy is safe and effective in improving growth and fat-soluble vitamin absorption in children and adolescents with inborn errors of bile acid metabolism due to amidation defects. (Hepatology 2015;61:268-274)

Energy storage devices (ESD), such as UPS batteries, have been repurposed in datacenter as a promising tuning knob for peak power shaving and power cost reducing. However, batteries progressively aging due to irregular usage patterns, which result in less effective capacity and even pose serious threat to server availability. Nevertheless, prior proposals largely ignore the aging issues of battery which may lead to low energy availability for datacenter servers. To fill this critical void, we thoroughly investigate battery aging on a heavily instrumented prototype system over an observation period of ten months. We propose Battery Anti-Aging Treatment Plus (BAAT-P), a novel power delivery architecture included aging management algorithms from the perspective of computing system to hide, reduce, mitigate and plan the battery aging effects for high energy availability in datacenter. Our techniques exploit diverse battery aging mechanisms and dynamic aging management algorithms to provide system-level availability guarantee for datacenter. We evaluate the BAAT-P design with a real prototype. Compared with a battery powered datacenter without aging management policies, the results show that BAAT-P can extend battery lifetime by 72 percent, reduce battery cost by 33 percent and effectively improve energy availability for datacenter servers while maintaining workload performance for the performance critical workloads.