{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,29]],"date-time":"2026-10-29T22:54:35Z","timestamp":1793314475122,"version":"4.5.0"},"reference-count":15,"publisher":"American Association for the Advancement of Science (AAAS)","issue":"5988","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Science"],"published-print":{"date-parts":[[2010,7,9]]},"abstract":"Correcting a Liver Problem<\/jats:title>\n \n The classical form of \u03b1\n 1<\/jats:sub>\n -antitrypsin (AT) deficiency is caused by a point mutation that alters the folding and causes intracellular aggregation of AT\u2014an abundant liver-derived plasma glycoprotein. AT deficiency is the most common genetic cause of liver disease in childhood and can also lead to cirrhosis and\/or hepatocellular carcinoma in adulthood. Carbamazepine is a drug known to be well tolerated in humans that enhances the intracellular degradation process known as autophagy. Now,\n \n Hidvegi\n et al.<\/jats:italic>\n <\/jats:bold>\n (p.\n 229<\/jats:related-article>\n , published online June 3; see the Perspective by\n \n Sifers<\/jats:related-article>\n <\/jats:bold>\n ) show that carbamazepine can reduce the severity of liver disease in a mouse model of AT deficiency by enhancing the degradation of misfolded accumulated AT.\n <\/jats:p>","DOI":"10.1126\/science.1190354","type":"journal-article","created":{"date-parts":[[2010,6,3]],"date-time":"2010-06-03T22:47:00Z","timestamp":1275605220000},"page":"229-232","source":"Crossref","is-referenced-by-count":562,"title":["An Autophagy-Enhancing Drug Promotes Degradation of Mutant \u03b1\n 1<\/sub>\n -Antitrypsin Z and Reduces Hepatic Fibrosis"],"prefix":"10.1126","volume":"329","author":[{"given":"Tunda","family":"Hidvegi","sequence":"first","affiliation":[{"name":"Departments of Pediatrics, Pathology, Cell Biology and Physiology, University of Pittsburgh School of Medicine, Pittsburgh, PA 15261 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