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Start Over Author "FAA, G." Publisher elsevier b.v.
21 results on '"FAA, G."'

5. HPLC-ESI-MS top-down analysis of salivary peptides of preterm newborns evidenced high activity of some exopeptidases and convertases during late fetal development

Author

Boroumand, M., Iavarone, F., Manconi, B., Pieroni, L., Greco, V., Vento, G., Tirone, C., Desiderio, C., Fiorita, A., Faa, G., Messana, I., Cabras, T., Olianas, A., Castagnola, M., Greco V. (ORCID:0000-0003-4521-0020), Boroumand, M., Iavarone, F., Manconi, B., Pieroni, L., Greco, V., Vento, G., Tirone, C., Desiderio, C., Fiorita, A., Faa, G., Messana, I., Cabras, T., Olianas, A., Castagnola, M., and Greco V. (ORCID:0000-0003-4521-0020)

Abstract

To have information on the proteolytic activity of convertases and exo-peptidases on human salivary proteins, this study investigated the relative amounts of the truncated proteoforms in the saliva of preterm newborns and compared them with the relative amounts measured in saliva of at-term newborns, of babies (0–10 years old) and of adults. Results indicated that convertase(s), acting on acidic proline-rich proteins and histatin 3, and carboxypeptidase(s) acting on acidic proline-rich proteins, P–C peptide, histatin 6 and statherin were many folds more active in preterm newborns than in the other groups. Conversely, the aminopeptidase responsible for the removal of the N-terminal Asp residue of statherin was not active in preterm newborns, becoming active only several months after the normal term of delivery. The high activity of convertases determined in preterm newborns suggests that it is required for the molecular events connected to the fetus development, and encourages further studies devoted to the characterization of their specific substrates.

Published
2021

13. Liver pathology in Wilson's disease: From copper overload to cirrhosis.

Author

Gerosa, C., Fanni, D., Congiu, T., Piras, M., Cau, F., Moi, M., and Faa, G.

Subjects
*HEPATOLENTICULAR degeneration, *LIVER physiology, *CIRRHOSIS of the liver, *TRANSMISSION electron microscopy, *MITOCHONDRIAL physiology
Abstract

Abstract Wilson's disease (WD) is a genetic metabolic disease strictly associated with liver cirrhosis. In this review, the genetic bases of the disease are discussed, with emphasis on the role of ATP7B (the Wilson disease protein) dysfunction as a determinant factor of systemic copper overload. Regarding the different multiple mutations described in WD patients, the peculiarity of Sardinian population is highlighted, Sardinians carrying a rare deletion in the promoter (5′ UTR) of the WD gene. The role of epigenetic changes in the clinical presentation and evolution of liver disease in WD patients is also discussed, nutrition probably representing a relevantly risk factor in WD patients. The role of transmission electron microscopy in the diagnosis of WD-related liver disease is underlined. Mitochondrial changes, increased peroxisomes fat droplets, lipolysosomes and intranuclear glycogen inclusions are reported as the most frequent ultrastructural changes in the liver of WD carriers. The role of histochemical stains for copper is analyzed, and the Timm's method is suggested as the most sensitive one for revealing hepatic copper overload in all stage of WD. The marked variability of the histological liver changes occurring in WD is underlined simple steatosis may represent the only pathological changes, frequently associated with glycogenated nuclei. Mallory-Denk bodies lipogranulomas alcoholic and non-alcoholic fatty liver disease ending with bridging fibrosis and cirrhosis. Finally, the reversal of fibrosis as a possible therapeutic objective in WD is discussed. Graphical abstract This article highlights the molecular mechanisms underlying copper trafficking inside the hepatocyte, focusing on the role of hCtr1 (the copper transporter 1) and the copper metallochaperone Atox1 in copper transfer from the sinusoidal lumen toward the Golgi Apparatus where the ATP7B (Wilson disease protein) is located. Unlabelled Image Highlights • Altered copper metabolism and liver overload • Open questions regarding the relationships between copper storage and liver disease • Morphology role is to identify the structural and ultrastructural aspects. • Liver histology and Wilson disease, non-alcoholic fatty liver disease, viral hepatitis [ABSTRACT FROM AUTHOR]

Published
2019
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