Iron misregulation and neurodegenerative disease in mouse models that lack iron regulatory proteins

Neurobiology of Disease
Manik C GhoshTracey A Rouault

Abstract

Iron regulatory proteins 1 and 2 (IRP1 and IRP2) are two cytosolic proteins that maintain cellular iron homeostasis by binding to RNA stem loops known as iron responsive elements (IREs) that are found in the untranslated regions of target mRNAs that encode proteins involved in iron metabolism. IRPs modify the expression of iron metabolism genes, and global and tissue-specific knockout mice have been made to evaluate the physiological significance of these iron regulatory proteins (Irps). Here, we will discuss the results of the studies that have been performed with mice engineered to lack the expression of one or both Irps and made in different strains using different methodologies. Both Irp1 and Irp2 knockout mice are viable, but the double knockout (Irp1(-/-)Irp2(-/-)) mice die before birth, indicating that these Irps play a crucial role in maintaining iron homeostasis. Irp1(-/-) mice develop polycythemia and pulmonary hypertension, and when these mice are challenged with a low iron diet, they die early of abdominal hemorrhages, suggesting that Irp1 plays an essential role in erythropoiesis and in the pulmonary and cardiovascular systems. Irp2(-/-) mice develop microcytic anemia, erythropoietic protoporphyria and a progressiv...Continue Reading

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Citations

Feb 19, 2016·Journal of Alzheimer's Disease : JAD·He XuPaul A Adlard
Mar 31, 2016·Current Opinion in Genetics & Development·Tracey A Rouault
Jun 14, 2016·Parkinson's Disease·Yorka MuñozMarco T Núñez
Apr 25, 2012·PloS One·Guangwei DuXuemei Huang
Feb 5, 2013·Free Radical Biology & Medicine·Orly WeinrebTamar Amit
Sep 3, 2013·Experimental Gerontology·Keith C DeRuisseauRobert P Doyle
Nov 7, 2015·Neurobiology of Disease·Elias Aizenman, Pier G Mastroberardino
Mar 17, 2015·Neurobiology of Disease·Manik C GhoshTracey A Rouault
Feb 27, 2018·The Journal of Clinical Investigation·Manik C GhoshTracey A Rouault
Mar 28, 2019·Brain : a Journal of Neurology·Gregory CostainGrace Yoon
Jun 28, 2019·Brain : a Journal of Neurology·Monica S CooperDavid J Amor
Jun 28, 2019·Brain : a Journal of Neurology·Nunziata MaioTracey A Rouault
Oct 30, 2019·The Journal of Clinical Investigation·Veena SangkhaeElizabeta Nemeth
Sep 10, 2019·The Journal of Clinical Investigation·Nathanael J LeeDaniel S Reich
May 2, 2019·Proceedings of the National Academy of Sciences of the United States of America·Huihui LiKuanyu Li
Aug 21, 2020·Learning & Memory·Jack T Rogers, Catherine M Cahill
Mar 30, 2019·Biometals : an International Journal on the Role of Metal Ions in Biology, Biochemistry, and Medicine·Tracey A Rouault
Apr 24, 2021·Journal of Biomedical Science·Nan SunJinquan Cai
Aug 28, 2021·Life Sciences·Dapinder KaurSimona Bungau
Oct 2, 2021·Proceedings of the National Academy of Sciences of the United States of America·Jin-Seon YookSoonkyu Chung

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