The mitochondrial uncoupling protein 2 gene is causal for the spontaneous polycystic liver diseases in mice

Misa Hirose; Paul Schilf; Sarah Rohde; Yask Gupta; Tiphaine Sancerni; Marie Clotilde Alves-Guerra; Christian Sina; Robert Jaster; Bruno Miroux; Saleh M. Ibrahim

doi:10.1016/j.mito.2017.10.011

The mitochondrial uncoupling protein 2 gene is causal for the spontaneous polycystic liver diseases in mice

Misa Hirose, Paul Schilf, Sarah Rohde, Yask Gupta, Tiphaine Sancerni, Marie Clotilde Alves-Guerra, Christian Sina, Robert Jaster, Bruno Miroux, Saleh M. Ibrahim^*

^*Korrespondierende/r Autor/-in für diese Arbeit

1 Zitat (Scopus)

Abstract

Polycystic liver diseases (PCLDs) are autosomal dominant disorders. To date, 3 genes are known to be associated with the disease, SEC63 and PRKCSH and LRP5. Here, we report that mice deficient in the mitochondrial uncoupling protein 2 gene (Ucp2^−/−) spontaneously developed PCLDs when they were over 12 months old. Macroscopical observation, blood chemistry as well as histopathological analysis demonstrated the PCLDs found in Ucp2^−/− mice were very similar to the findings in human PCLDs. This is the first report describing the gene encoding mitochondrial protein is causative for PCLDs. UCP2 may be a biomarker of the PCLDs in humans.

Originalsprache	Englisch
Zeitschrift	Mitochondrion
Jahrgang	42
Seiten (von - bis)	50-53
Seitenumfang	4
ISSN	1567-7249
DOIs	https://doi.org/10.1016/j.mito.2017.10.011
Publikationsstatus	Veröffentlicht - 01.09.2018

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The authors thank Miriam Freitag, Stephanie Wichmann, Miriam Daumann, Ann-Kathrin Brethack, and Heidi Schlichting for excellent technical support. The authors also thank Daniel Gotthardt for his critical reading of the manuscript. This work was supported by grants from the Bundesministerium für Bildung und Forschung (BMBF, 0315892A and 0315892B ) and the University of Lübeck ( P01-2012 ). Appendix A

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title = "The mitochondrial uncoupling protein 2 gene is causal for the spontaneous polycystic liver diseases in mice",

abstract = "Polycystic liver diseases (PCLDs) are autosomal dominant disorders. To date, 3 genes are known to be associated with the disease, SEC63 and PRKCSH and LRP5. Here, we report that mice deficient in the mitochondrial uncoupling protein 2 gene (Ucp2−/−) spontaneously developed PCLDs when they were over 12 months old. Macroscopical observation, blood chemistry as well as histopathological analysis demonstrated the PCLDs found in Ucp2−/− mice were very similar to the findings in human PCLDs. This is the first report describing the gene encoding mitochondrial protein is causative for PCLDs. UCP2 may be a biomarker of the PCLDs in humans.",

author = "Misa Hirose and Paul Schilf and Sarah Rohde and Yask Gupta and Tiphaine Sancerni and Alves-Guerra, \{Marie Clotilde\} and Christian Sina and Robert Jaster and Bruno Miroux and Ibrahim, \{Saleh M.\}",

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doi = "10.1016/j.mito.2017.10.011",

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T1 - The mitochondrial uncoupling protein 2 gene is causal for the spontaneous polycystic liver diseases in mice

AU - Hirose, Misa

AU - Schilf, Paul

AU - Rohde, Sarah

AU - Gupta, Yask

AU - Sancerni, Tiphaine

AU - Alves-Guerra, Marie Clotilde

AU - Sina, Christian

AU - Jaster, Robert

AU - Miroux, Bruno

AU - Ibrahim, Saleh M.

PY - 2018/9/1

Y1 - 2018/9/1

N2 - Polycystic liver diseases (PCLDs) are autosomal dominant disorders. To date, 3 genes are known to be associated with the disease, SEC63 and PRKCSH and LRP5. Here, we report that mice deficient in the mitochondrial uncoupling protein 2 gene (Ucp2−/−) spontaneously developed PCLDs when they were over 12 months old. Macroscopical observation, blood chemistry as well as histopathological analysis demonstrated the PCLDs found in Ucp2−/− mice were very similar to the findings in human PCLDs. This is the first report describing the gene encoding mitochondrial protein is causative for PCLDs. UCP2 may be a biomarker of the PCLDs in humans.

AB - Polycystic liver diseases (PCLDs) are autosomal dominant disorders. To date, 3 genes are known to be associated with the disease, SEC63 and PRKCSH and LRP5. Here, we report that mice deficient in the mitochondrial uncoupling protein 2 gene (Ucp2−/−) spontaneously developed PCLDs when they were over 12 months old. Macroscopical observation, blood chemistry as well as histopathological analysis demonstrated the PCLDs found in Ucp2−/− mice were very similar to the findings in human PCLDs. This is the first report describing the gene encoding mitochondrial protein is causative for PCLDs. UCP2 may be a biomarker of the PCLDs in humans.

UR - https://www.scopus.com/pages/publications/85034784780

U2 - 10.1016/j.mito.2017.10.011

DO - 10.1016/j.mito.2017.10.011

M3 - Journal articles

AN - SCOPUS:85034784780

SN - 1567-7249

VL - 42

SP - 50

EP - 53

JO - Mitochondrion

JF - Mitochondrion

ER -

The mitochondrial uncoupling protein 2 gene is causal for the spontaneous polycystic liver diseases in mice

Abstract

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