Findings in the Area of Alpha 1-Antitrypsin Deficiency Reported from University of Florida (Quantitative Measurement of the Histo-logical Features of Alpha-1 Antitrypsin Deficiency-associated Liver Disease In Biopsy Specimens)

摘要

2021 SEP 21 (NewsRx) - By a News Reporter-Staff News Editor at Disease Prevention Daily - Investigators publish new report on Connective Tissue Diseases and Conditions - Alpha 1-Antitrypsin Deficiency. According to news reporting originating from Gainesville, Florida, by NewsRx correspondents, research stated, "Pathological mutations in Alpha-1 Antitrypsin (AAT) protein cause retention of toxic polymers in the hepatocyte endoplasmic reticulum. The risk for cirrhosis in AAT deficiency is likely directly related to retention of these polymers within the liver." Funders for this research include Alpha-1 Foundation, National Center for Advancing Translational Sciences, National Center for Advancing Translational Sciences. Our news editors obtained a quote from the research from the University of Florida, "Polymers are classically identified on liver biopsy as inclusion bodies by periodic acid schiff staining after diastase treatment and immunohistochemistry However, characterization of the polymer burden within a biopsy sample is limited to a semi-quantitative scale as described by a pathologist. Better methods to quantify polymer are needed to advance our understanding of pathogenesis of disease. Therefore, we developed a method to quantify polymer aggregation from standard histologic specimens. In addition, we sought to understand the relationship of polymer burden and other histologic findings to the presence of liver fibrosis.