Genetic Determined Iron Starvation Signature in Friedreich's Ataxia

Background Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. Objectives The objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients. Methods In...

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Vydané v:	Movement disorders Ročník 39; číslo 7; s. 1088 - 1098
Hlavní autori:	Grander, Manuel, Haschka, David, Indelicato, Elisabetta, Kremser, Christian, Amprosi, Matthias, Nachbauer, Wolfgang, Henninger, Benjamin, Stefani, Ambra, Högl, Birgit, Fischer, Christine, Seifert, Markus, Kiechl, Stefan, Weiss, Günter, Boesch, Sylvia
Médium:	Journal Article
Jazyk:	English
Vydavateľské údaje:	Hoboken, USA John Wiley & Sons, Inc 01.07.2024 Wiley Subscription Services, Inc
Predmet:	Ataxia Divalent metal transporter-1 Down-regulation Erythropoiesis Ferritin Friedreich's ataxia Hemoglobin Hepatocytes Hepcidin Intracellular Iron Iron deficiency Leukocytes (mononuclear) Liver Magnetic resonance imaging Movement disorders MRI relaxometry mRNA Peripheral blood mononuclear cells Regulatory proteins Spleen Transferrins Friedreich's ataxia iron hepcidin MRI relaxometry liver
ISSN:	0885-3185, 1531-8257, 1531-8257
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Abstract	Background Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. Objectives The objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients. Methods In FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R2‐relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA‐expansion (GAA1). Results We recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1‐repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R2‐relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R2* values inversely correlated with the GAA1‐repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter‐1 mRNA, particularly in patients with >500 GAA1‐repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria. Conclusions We provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
AbstractList	Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking.BACKGROUNDEarly studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking.The objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients.OBJECTIVESThe objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients.In FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R2-relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA-expansion (GAA1).METHODSIn FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R2-relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA-expansion (GAA1).We recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1-repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R2-relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R2 values inversely correlated with the GAA1-repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter-1 mRNA, particularly in patients with >500 GAA1-repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria.RESULTSWe recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1-repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R2-relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R2 values inversely correlated with the GAA1-repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter-1 mRNA, particularly in patients with >500 GAA1-repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria.We provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.CONCLUSIONSWe provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. The objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients. In FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R -relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA-expansion (GAA1). We recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1-repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R -relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R * values inversely correlated with the GAA1-repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter-1 mRNA, particularly in patients with >500 GAA1-repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria. We provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. BackgroundEarly studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking.ObjectivesThe objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients.MethodsIn FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R2‐relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA‐expansion (GAA1).ResultsWe recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1‐repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R2‐relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R2* values inversely correlated with the GAA1‐repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter‐1 mRNA, particularly in patients with >500 GAA1‐repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria.ConclusionsWe provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. Background Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. Objectives The objective is to characterize systemic iron metabolism, body iron storages, and intracellular iron regulation in FA patients. Methods In FA patients and matched healthy controls, we assessed serum iron parameters, regulatory hormones as well as the expression of regulatory proteins and iron distribution in peripheral blood mononuclear cells (PBMCs). We applied magnetic resonance imaging with R2‐relaxometry to quantify iron storages in the liver, spleen, and pancreas. Across all evaluations, we assessed the influence of the genetic severity as expressed by the length of the shorter GAA‐expansion (GAA1). Results We recruited 40 FA patients (19 women). Compared to controls, FA patients displayed lower serum iron and transferrin saturation. Serum ferritin, hepcidin, mean corpuscular hemoglobin and mean corpuscular volume in FA inversely correlated with the GAA1‐repeat length, indicating iron deficiency and restricted availability for erythropoiesis with increasing genetic severity. R2‐relaxometry revealed a reduction of splenic and hepatic iron stores in FA. Liver and spleen R2* values inversely correlated with the GAA1‐repeat length. FA PBMCs displayed downregulation of ferritin and upregulation of transferrin receptor and divalent metal transporter‐1 mRNA, particularly in patients with >500 GAA1‐repeats. In FA PBMCs, intracellular iron was not increased, but shifted toward mitochondria. Conclusions We provide evidence for a previously unrecognized iron starvation signature at systemic and cellular levels in FA patients, which is related to the underlying genetic severity. These findings challenge the use of systemic iron lowering therapies in FA. © 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society.
Author	Kiechl, Stefan Amprosi, Matthias Boesch, Sylvia Grander, Manuel Haschka, David Henninger, Benjamin Weiss, Günter Seifert, Markus Kremser, Christian Högl, Birgit Indelicato, Elisabetta Fischer, Christine Nachbauer, Wolfgang Stefani, Ambra
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Copyright	2024 The Authors. published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. 2024 The Authors. Movement Disorders published by Wiley Periodicals LLC on behalf of International Parkinson and Movement Disorder Society. 2024. This article is published under http://creativecommons.org/licenses/by-nc-nd/4.0/ (the “License”). Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
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Notes	Manuel Grander and David Haschka contributed equally as first authors. Günter Weiss and Sylvia Boesch contributed equally as last authors. The authors declare that there are no conflicts of interest to report related to this work. Friedreich's Ataxia Research Alliance (FARA), FARA Ireland and FARA Australia. Funding agencies Relevant Conflicts of interst/financial disclosures ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 14 content type line 23
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Snippet	Background Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. Objectives The... Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking. The objective is to... BackgroundEarly studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking.ObjectivesThe... Early studies in cellular models suggested an iron accumulation in Friedreich's ataxia (FA), yet findings from patients are lacking.BACKGROUNDEarly studies in...
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SubjectTerms	Ataxia Divalent metal transporter-1 Down-regulation Erythropoiesis Ferritin Friedreich's ataxia Hemoglobin Hepatocytes Hepcidin Intracellular Iron Iron deficiency Leukocytes (mononuclear) Liver Magnetic resonance imaging Movement disorders MRI relaxometry mRNA Peripheral blood mononuclear cells Regulatory proteins Spleen Transferrins
Title	Genetic Determined Iron Starvation Signature in Friedreich's Ataxia
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