Exosomal MicroRNAs as Potential Biomarkers of Hepatic Injury and Kidney Disease in Glycogen Storage Disease Type Ia Patients

Glycogen storage disease type Ia (GSDIa) is an inherited metabolic disorder caused by mutations in the enzyme glucose-6-phosphatase-α (G6Pase-α). Affected individuals develop renal and liver complications, including the development of hepatocellular adenoma/carcinoma and kidney failure. The purpose...

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Detalles Bibliográficos
Autores principales: Resaz, Roberta, Cangelosi, Davide, Segalerba, Daniela, Morini, Martina, Uva, Paolo, Bosco, Maria Carla, Banderali, Giuseppe, Estrella, Ana, Wanner, Corbinian, Weinstein, David A., Sechi, Annalisa, Paci, Sabrina, Melis, Daniela, Di Rocco, Maja, Lee, Young Mok, Eva, Alessandra
Formato: Online Artículo Texto
Lenguaje:English
Publicado: MDPI 2021
Materias:
Article
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8745197/
https://www.ncbi.nlm.nih.gov/pubmed/35008754
http://dx.doi.org/10.3390/ijms23010328
Descripción
Sumario:Glycogen storage disease type Ia (GSDIa) is an inherited metabolic disorder caused by mutations in the enzyme glucose-6-phosphatase-α (G6Pase-α). Affected individuals develop renal and liver complications, including the development of hepatocellular adenoma/carcinoma and kidney failure. The purpose of this study was to identify potential biomarkers of the evolution of the disease in GSDIa patients. To this end, we analyzed the expression of exosomal microRNAs (Exo-miRs) in the plasma exosomes of 45 patients aged 6 to 63 years. Plasma from age-matched normal individuals were used as controls. We found that the altered expression of several Exo-miRs correlates with the pathologic state of the patients and might help to monitor the progression of the disease and the development of late GSDIa-associated complications.

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