Liver organoids reproduce alpha-1 antitrypsin deficiency-related liver disease
نویسندگان
چکیده
منابع مشابه
Liver disease and alpha 1-antitrypsin deficiency.
Discovery of AAT deficiency by Laurell and Eriksson in 1963 [2] provided a foundation for current thinking about the pathogenesis of pulmonary emphysema [3,4]. Although AAT deficiency has become one of the best understood genetic disorders at a molecular and protein level, many questions about the clinical disease remain unanswered. Current American and International research projects should pr...
متن کاملAlpha 1-antitrypsin deficiency, complement activation, and chronic liver disease.
Activation of the complement system, the main humoral mediator of inflammation, is restrained by the action of enzyme inhibitors including alpha 1-antitrypsin. Deficiency leads to chronic liver disease in about one in five children with this genetic defect. Complement activation was investigated in 34 children with alpha 1 AT deficiency (12 with minimal, 10 with moderate, and 12 with severe liv...
متن کاملLiver transplantation for advanced liver disease with alpha-1-antitrypsin deficiency.
ALPHA-I-antitrypsin deficiency associated with n chronic obstructive airway disease was recognized in 1963 by Laurell and Ericksson. I In 1969, . Sharp' described the first cases of alpha-l-antitrypsin-deficiency disease in children with cirrhosis. Since then, this ·inborn error has been recognized as one of the more common factors in cirrhosis of infancy and childhood,] including "neonatal hep...
متن کاملAlpha-1 antitrypsin deficiency among Indian children with liver disorders.
AIMS To determine the frequency of alpha-1 antitrypsin (AAT) deficiency in children with chronic liver disease (CLD) and neonatal cholestasis syndrome (NCS). METHODS All children with NCS (n=23) or CLD (n=35) attending the Pediatric Gastroenterology Clinic between November 2003 and July 2005 were screened for AAT deficiency using phenotyping through isoelectric focusing of plasma. RESULTS O...
متن کاملAlpha-1-antitrypsin phenotypes in adult liver disease patients
Alpha-1-antitrypsin (AAT) is an important serine protease inhibitor in humans. Hereditary alpha-1-antitrypsin deficiency (AATD) affects lungs and liver. Liver disease caused by AATD in paediatric patients has been previously well documented. However, the association of liver disease with alpha-1-antitrypsin gene polymorphisms in adults is less clear. Therefore, we aimed to study AAT polymorphis...
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ژورنال
عنوان ژورنال: Hepatology International
سال: 2019
ISSN: 1936-0533,1936-0541
DOI: 10.1007/s12072-019-10007-y