Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression

Heterozygous mutations in the UBIAD1 gene cause Schnyder corneal dystrophy characterized by abnormal cholesterol and phospholipid deposits in the cornea. Ubiad1 protein was recently identified as Golgi prenyltransferase responsible for biosynthesis of vitamin K2 and CoQ10, a key protein in the mitoc...

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Autores principales: Morales, Carlos R., Grigoryeva, Lubov S., Pan, Xuefang, Bruno, Luigi, Hickson, Gilles, Ngo, Michael H., McMaster, Christopher R., Samuels, Mark E., Pshezhetsky, Alexey V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2014
Materias:
Short Communication
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5121353/
https://www.ncbi.nlm.nih.gov/pubmed/27896114
http://dx.doi.org/10.1016/j.ymgmr.2014.09.001
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author Morales, Carlos R.
Grigoryeva, Lubov S.
Pan, Xuefang
Bruno, Luigi
Hickson, Gilles
Ngo, Michael H.
McMaster, Christopher R.
Samuels, Mark E.
Pshezhetsky, Alexey V.
author_facet Morales, Carlos R.
Grigoryeva, Lubov S.
Pan, Xuefang
Bruno, Luigi
Hickson, Gilles
Ngo, Michael H.
McMaster, Christopher R.
Samuels, Mark E.
Pshezhetsky, Alexey V.
author_sort Morales, Carlos R.
collection PubMed
description Heterozygous mutations in the UBIAD1 gene cause Schnyder corneal dystrophy characterized by abnormal cholesterol and phospholipid deposits in the cornea. Ubiad1 protein was recently identified as Golgi prenyltransferase responsible for biosynthesis of vitamin K2 and CoQ10, a key protein in the mitochondrial electron transport chain. Our study shows that silencing UBIAD1 in cultured human hepatocellular carcinoma cells causes dramatic morphological changes and cholesterol storage in the mitochondria, emphasizing an important role of UBIAD1 in mitochondrial function.
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spelling pubmed-51213532016-11-28 Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression Morales, Carlos R. Grigoryeva, Lubov S. Pan, Xuefang Bruno, Luigi Hickson, Gilles Ngo, Michael H. McMaster, Christopher R. Samuels, Mark E. Pshezhetsky, Alexey V. Mol Genet Metab Rep Short Communication Heterozygous mutations in the UBIAD1 gene cause Schnyder corneal dystrophy characterized by abnormal cholesterol and phospholipid deposits in the cornea. Ubiad1 protein was recently identified as Golgi prenyltransferase responsible for biosynthesis of vitamin K2 and CoQ10, a key protein in the mitochondrial electron transport chain. Our study shows that silencing UBIAD1 in cultured human hepatocellular carcinoma cells causes dramatic morphological changes and cholesterol storage in the mitochondria, emphasizing an important role of UBIAD1 in mitochondrial function. Elsevier 2014-09-19 /pmc/articles/PMC5121353/ /pubmed/27896114 http://dx.doi.org/10.1016/j.ymgmr.2014.09.001 Text en © 2014 The Authors http://creativecommons.org/licenses/by-nc-nd/3.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/3.0/).
spellingShingle Short Communication
Morales, Carlos R.
Grigoryeva, Lubov S.
Pan, Xuefang
Bruno, Luigi
Hickson, Gilles
Ngo, Michael H.
McMaster, Christopher R.
Samuels, Mark E.
Pshezhetsky, Alexey V.
Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression
title Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression
title_full Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression
title_fullStr Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression
title_full_unstemmed Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression
title_short Mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of UBIAD1 gene expression
title_sort mitochondrial damage and cholesterol storage in human hepatocellular carcinoma cells with silencing of ubiad1 gene expression
topic Short Communication
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5121353/
https://www.ncbi.nlm.nih.gov/pubmed/27896114
http://dx.doi.org/10.1016/j.ymgmr.2014.09.001
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