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Name(s): John Wen High School(s): West High School, Iowa City, IA Mentor: Dr. Kevin Glenn

Name(s): John Wen High School(s): West High School, Iowa City, IA Mentor: Dr. Kevin Glenn Project Title: A Novel Lectin-Like Ubiquitin Ligase Degrades Disease- Causing A1AT-Z.

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Name(s): John Wen High School(s): West High School, Iowa City, IA Mentor: Dr. Kevin Glenn

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  1. Name(s): John Wen High School(s): West High School, Iowa City, IA Mentor: Dr. Kevin Glenn Project Title: A Novel Lectin-Like Ubiquitin Ligase Degrades Disease- Causing A1AT-Z The most common cause of childhood liver failure and one of the three most common lethal genetic diseases in Caucasians is A1AT deficiency. The term A1AT-Z deficiency is a misleading term; mutant A1AT-Z accumulates in the liver of A1AT deficiency patients causing liver scarring, inflammation, and carcinoma. The accumulation then results in reduced secretion into the blood and the lung. We examine the degradation of A1AT-Z in liver cell lines to see how it is cleared. We identified the novel lectin-like E3 ubiquitin ligases FBG1 and FBG2 that participate in the clearance of A1AT-Z. Furthermore, we show that soluble A1AT-Z is degraded by the ubiquitin proteasome system and by autophagy while the insoluble form of A1AT-Z is predominately degraded by autophagy. FBG1, FBG2, and A1AT-Z are all found in the liver, in the same cellular compartment, and they directly interact as shown by immunoprecipitation. Overexpression of FBG1 and FBG2 decreases the half-life of A1AT-Z from 13 hours to 4 hours and 10 hours respectively. Finally, we show that FBG1 may increase A1AT-Z secretion, suggesting that FBG1 may be used to treat both lung and liver disease in A1AT-Z deficient patients.

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