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dc.contributor.authorCortez, Cristián [Univ Mayor, Fac Sci, Ctr Genom & Bioinformat, Santiago 8580745, Chile]es_CL
dc.contributor.authorKlein, Andrés D.es_CL
dc.contributor.authorEsteban Oyarzún, Juanes_CL
dc.contributor.authorZanlungo, Silvanaes_CL
dc.date.accessioned2020-04-08T14:11:55Z
dc.date.accessioned2020-04-13T18:12:46Z
dc.date.available2020-04-08T14:11:55Z
dc.date.available2020-04-13T18:12:46Z
dc.date.issued2018es_CL
dc.identifier.citationKlein, A. D., Oyarzún, J. E., Cortez, C., & Zanlungo, S. (2018). Gadolinium Chloride Rescues Niemann–Pick Type C Liver Damage. International journal of molecular sciences, 19(11), 3599.es_CL
dc.identifier.issn1422-0067es_CL
dc.identifier.urihttps://doi.org/10.3390/ijms19113599es_CL
dc.identifier.urihttp://repositorio.umayor.cl/xmlui/handle/sibum/6217
dc.description.abstractNiemann-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 CD68A marker of Kupffer cells in the liverand 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.es_CL
dc.description.sponsorshipFondecytComision Nacional de Investigacion Cientifica y Tecnologica (CONICYT)CONICYT FONDECYT [1180337, 1150186]; CONICYT-PCHA/Doctorado-Nacional/grant [2014-62968]; European Union's Horizon 2020 research and innovation programme (RISE) under the Marie Sklodowska-Curie grant [734825]es_CL
dc.description.sponsorshipThis research was funded by Fondecyt (grant #1180337 to A.D.K. and #1150186 to S.Z.), CONICYT-PCHA/Doctorado-Nacional/grant #2014-62968 to J.E.O. and by the European Union's Horizon 2020 research and innovation programme (RISE) under the Marie Sklodowska-Curie grant agreement #.734825.es_CL
dc.language.isoenes_CL
dc.publisherMDPIes_CL
dc.rightsAttribution-NonCommercial-NoDerivs 3.0 Chile
dc.sourceInt. J. Mol. Sci., NOV 2018. 19(11)
dc.subjectBiochemistry & Molecular Biology; Chemistry, Multidisciplinaryes_CL
dc.titleGadolinium Chloride Rescues Niemann-Pick Type C Liver Damagees_CL
dc.typeArtículoes_CL
umayor.facultadCIENCIASes_CL
umayor.politicas.sherpa/romeoDOAJ Gold, Green Publishedes_CL
umayor.indexadoWOS:000451528500315es_CL
umayor.indexadoPMID: 30441844es_CL
dc.identifier.doiDOI: 10.3390/ijms19113599es_CL]
umayor.indicadores.wos-(cuartil)Q2es_CL
umayor.indicadores.scopus-(scimago-sjr)SCIMAGO/ INDICE H: 114 Hes_CL


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