KLEIN POSTERNACK, ANDRES DAVID
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KLEIN POSTERNACK, ANDRES DAVID
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andresklein@udd.cl
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Scopus Author ID
46062217700

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2021 2021 2020 2020 2019 2019 2018 2018 0.0 0.0 0.2 0.2 0.4 0.4 0.6 0.6 0.8 0.8 1.0 1.0

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Publication

Identification of genetic modifiers of murine hepatic β-glucocerebrosidase activity

2021 , Anyelo Durán , Boris Rebolledo-Jaramillo , Valeria Olguin , Marcelo Rojas-Herrera , Macarena Las Heras , CALDERON GIADROSIC, JUAN FRANCISCO , Silvana Zanlungo , David A. Priestman , Frances M. Platt , KLEIN POSTERNACK, ANDRES DAVID

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Gadolinium Chloride Rescues Niemann–Pick Type C Liver Damage

2018 , KLEIN POSTERNACK, ANDRES DAVID , Juan OyarzĂşn , Cristian Cortez , Silvana Zanlungo

Niemann–Pick type C (NPC) disease is a rare neurovisceral cholesterol storage disorder that arises from loss of function mutations in the NPC1 or NPC2 genes. Soon after birth, some patients present with an aggressive hepatosplenomegaly and cholestatic signs. Histopathologically, the liver presents with large numbers of foam cells; however, their role in disease pathogenesis has not been explored in depth. Here, we studied the consequences of gadolinium chloride (GdCl3) treatment, a well-known Kupffer/foam cell inhibitor, at late stages of NPC liver disease and compared it with NPC1 genetic rescue in hepatocytes in vivo. GdCl3 treatment successfully blocked the endocytic capacity of hepatic Kupffer/foam measured by India ink endocytosis, decreased the levels CD68—A marker of Kupffer cells in the liver—and normalized the transaminase levels in serum of NPC mice to a similar extent to those obtained by genetic Npc1 rescue of liver cells. Gadolinium salts are widely used as magnetic resonance imaging (MRI) contrasts. This study opens the possibility of targeting foam cells with gadolinium or by other means for improving NPC liver disease. Synopsis: Gadolinium chloride can effectively rescue some parameters of liver dysfunction in NPC mice and its potential use in patients should be carefully evaluated.

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c-Abl Inhibition Activates TFEB and Promotes Cellular Clearance in a Lysosomal Disorder

2020 , Pablo S. Contreras , Pablo J. Tapia , Lila González-Hódar , Ivana Peluso , Chiara Soldati , Gennaro Napolitano , Maria Matarese , Macarena Las Heras , Cristian Valls , Alexis Martinez , Elisa Balboa , Juan Castro , Nancy Leal , Frances M. Platt , Andrzej Sobota , Dominic Winter , KLEIN POSTERNACK, ANDRES DAVID , Diego L. Medina , Andrea Ballabio , Alejandra R. Alvarez , Silvana Zanlungo

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