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α1-Antitrypsin Deficiency: Situation in Spain and Development of a Screening Program

✍ Scribed by Cristian de la Roza; Beatriz Lara; Sara Vilá; Marc Miravitlles

Book ID
119628428
Publisher
Elsevier Science
Year
2006
Tongue
Spanish
Weight
188 KB
Volume
42
Category
Article
ISSN
0300-2896

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␣-1-Antitrypsin (␣1-AT) deficiency is the most common cause of metabolic pediatric liver disease. Hepatocellular injury is caused by toxicity of the mutant ␣-1-antitrypsin Z (␣1-ATZ) molecule retained within hepatocytes. In these studies, we used the PiZ transgenic mouse model of ␣1-AT deficiency to