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Bruno Maria Custode on Muck Rack

Bruno Maria Custode

As seen in: Nature, Texas Medical Center
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Articles

Polymeric alpha-1 antitrypsin perturbs Paneth cell proteostasis and exacerbates intestinal inflammation

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By Francesco Annunziata, JIng Lu, Claudia D’Agostino, Bruno Maria Custode, Gaetano Raiola, Rossella De Cegli, Eugenio Del Prete, Elena Polishchuk, Debora Compare, Gerardo Nardone, Francesco Neri, Nunzia Pastore, Irene Relvini, Giovanna Federico, Valentina Schiano, Veronica Maffia, Barbara De Marino, Carmen Ambrosio
| Nature Verified
Abstract Alpha-1 antitrypsin (AAT) is a serine protease inhibitor that protects tissue from neutrophil elastase and other proteases, particularly in the lung. Mutations in SERPINA1, including the Z mutation, lead to AAT deficiency (AATD), characterized by reduced circulating AAT and increased risk of pulmonary emphysema, liver cirrhosis, and hepatocellular carcinoma.
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Folliculin Depletion Results in Liver Cell Damage and Cholangiocarcinoma Through MIT/TFE Activation

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By Bruno Maria Custode
| Texas Medical Center
Cell Death & Differentiation 10.1038/s41418-025-01486-8 Post-print Animals, Cholangiocarcinoma, Mice, Basic Helix-Loop-Helix Leucine Zipper Transcription Factors, Male, Tumor Suppressor Proteins, Liver, Mice, Knockout, Bile Duct Neoplasms, Mechanistic Target of Rapamycin Complex 1, Humans, Female, Proto-Oncogene Proteins, Mice, Inbred C57BL yes Custode, Bruno Maria; Annunziata, Francesco; Dos Santos Matos, Felipe; et al., "Folliculin Depletion Results in Liver Cell Damage and...
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