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Center for Iron & Heme Disorders at the University of Utah

铁中心

基本信息

批准号：
10676099
负责人：
JAMES Eric COX
金额：
$ 67.28万
依托单位：
UNIVERSITY OF UTAH
依托单位国家：
美国
项目类别：
财政年份：
2016
资助国家：
美国
起止时间：
2016-08-05 至 2026-07-31
项目状态：
未结题

来源：
https://reporter.nih.gov/project-details/10676099
关键词：
Affect Anemia Area Assisted Living Facilities Award Binding Biochemical Biochemistry Biological Biological Assay Biological Process Biomedical Research Cell Respiration Centers for Disease Control and Prevention (U.S.)Cessation of life Clinical Research Clustered Regularly Interspaced Short Palindromic Repeats Collaborations Copper Cultured Cells DNA biosynthesis Detection Diamond-Blackfan anemia Disease Educational workshop Enzymes Equipment Erythropoiesis Faculty Fees and Charges Free Radicals Funding Gene Modified Gene Mutation Generations Genes Globin Goals Hematology Hematopoiesis Heme Heme Group Hemeproteins Hemoglobin Homeostasis Human Genetics Individual Institution Interest Group Iron Iron Overload Letters Liquid substance Medicine Mentors Metabolic Diseases Metabolic Pathway Metabolism Metabolite Interaction Metals Methods Molecular Mononuclear Mutation National Institute of Diabetes and Digestive and Kidney Diseases Non-Malignant Pathology Persons Phenotype Pilot Projects Play Porphyrias Porphyrins Postdoctoral Fellow Progress Reports Proteins Proteomics Reagent Recording of previous events Research Research Activity Research Design Research Personnel Research Project Grants Research Training Resources Role Scientist Series Services Source Sulfur Technology Training Transition Elements Universities Utah Visit base cofactor cost effective design genome editing heme biosynthesis human disease interest macromolecule medical schools meetings member metabolomics metal metabolism metal metabolism disorder multidisciplinary new technology next generation novel operation oxygen transport pre-doctoral programs protein metabolite stem stem cell biology symposium synergism transcription activator-like effector nucleases

项目摘要

Project Summary: The proposed University of Utah Cooperative Centers of Excellence in Hematology (CCEH) brings together 38 investigators whose research activities are focused on various aspects of iron and heme metabolism and non- malignant hematology. Iron plays an essential role in many biological processes including heme synthesis, oxygen transport, cellular respiration and DNA synthesis. Malregulation of iron homeostasis, either from deficiency or excess, results in disease. Heme is a key component of hemoglobin and other hemoproteins, but heme also plays a regulatory role in a number of metabolic pathways. Disorders of heme biosynthesis are responsible for an important group of human diseases, namely the porphyrias. The Utah Center for Iron and Heme Disorders (CIHD) will support the activities of a Research Base of 38 investigators whose research projects focus on the roles of iron, porphyrins and heme in eukaryotic metabolism. The activities of center members encompass both basic and clinical studies designed to identify disease mechanisms. To accomplish our goals, we propose an Administrative Core and four Biomedical Research Cores. The majority of the cores are already present and have been adjusted to meet the needs of the CIHD. These include: an Iron and Heme Core, which can assay and quantitate metals, porphyrins, heme biosynthetic enzymes and iron-binding and other proteins; a Metabolomics Core, which provides metabolomic phenotyping and molecular identification; a Mutation Generation and Detection Core, which provides cutting edge genome editing through CRISPR and TALEN reagents; and a new core in this renewal, the Protein-Metabolite Interactomics Core that provides proteomics services but adds the unique technology of identification of metabolites that interact with a protein and may post-translationally modify the protein in novel ways. The services provided by these cores will enable individual investigators to: 1) identify the role of genes in hematopoiesis or iron overload; 2) determine the effects of gene modification or mutations on metabolism in cultured cells or biological fluids; and 3) identify at the biochemical level the effect of mutations or conditions that affect iron and heme homeostasis on all levels. The Administrative Core will provide budgetary and scientific guidance to CIHD activities. Core recharge fees will be used to enhance and expand core operations. An Enrichment Program is designed for trainees and young investigators in the fields of nonmalignant hematology and for senior investigators who wish to enter this field. The Research Base is drawn from both the University of Utah and other institutions, with half of the members belonging to institutions outside of Utah. We have increased the Research base from 22 to 38 with the addition of ~4 investigators each year. The goals of the CIHD are to be a national resource for studies involving iron and heme and to inspire the next generation of investigators focused on iron, heme and nonmalignant hematology.

项目摘要：拟议中的犹他州大学血液学卓越合作中心（CCEH）汇集了38个其研究活动集中在铁和血红素代谢的各个方面以及非铁代谢的研究者。恶性血液学铁在许多生物过程中起着重要作用，包括血红素合成，氧气运输、细胞呼吸和DNA合成。铁稳态失调，无论是从不足或过量，都会导致疾病。血红素是血红蛋白和其他血红素蛋白的关键成分，但血红素还在许多代谢途径中起调节作用。血红素生物合成障碍是导致一组重要的人类疾病，即卟啉症。犹他州钢铁中心血红素障碍（CIHD）将支持38名研究人员的研究基地的活动，项目集中于铁、卟啉和血红素在真核生物代谢中的作用。中心活动成员包括旨在确定疾病机制的基础和临床研究。完成我们的目标，我们提出了一个行政核心和四个生物医学研究核心。大多数核心已经存在，并已进行调整，以满足CIHD的需要。其中包括：铁和血红素核心，它可以分析和定量金属，卟啉，血红素生物合成酶和铁结合，代谢组学核心，提供代谢组学表型分析和分子鉴定; 突变生成和检测核心，通过CRISPR提供尖端的基因组编辑， TALEN试剂;以及此次更新中的新核心，蛋白质-代谢物相互作用组学核心，蛋白质组学服务，但增加了识别与蛋白质相互作用的代谢物的独特技术并且可以以新的方式对蛋白质进行后修饰。这些核心提供的服务将使个体研究者：1）确定基因在造血或铁过载中的作用; 2）确定基因修饰或突变对培养细胞或生物流体中代谢的影响;以及3）鉴定生化水平突变或条件的影响，影响铁和血红素稳态的所有水平。行政核心将为CIHD活动提供预算和科学指导。核心充值费用将用于加强和扩大核心业务。一个强化计划是为学员设计的，非恶性血液学领域的年轻研究者和希望进入该领域的高级研究者领域研究基地来自犹他州大学和其他机构，其中一半来自属于犹他州以外机构的成员。我们将研究基地从22个增加到38个，每年增加约4名调查员。CIHD的目标是成为国家研究资源涉及铁和血红素，并激励下一代的研究人员专注于铁，血红素和非恶性血液学

项目成果

期刊论文数量（70）

专著数量（0）

科研奖励数量（0）

会议论文数量（0）

专利数量（0）

Loss of the mitochondrial protein Abcb10 results in altered arginine metabolism in MEL and K562 cells and nutrient stress signaling through ATF4.

DOI：
10.1016/j.jbc.2023.104877
发表时间：
2023-07
期刊：
JOURNAL OF BIOLOGICAL CHEMISTRY
影响因子：
4.8
作者：
Miljkovic, Marisa;Seguin, Alexandra;Jia, Xuan;Cox, James E;Catrow, Jonathan Leon;Bergonia, Hector;Phillips, John D;Stephens, W Zac;Ward, Diane M
通讯作者：
Ward, Diane M

Inhibition of the skeletal muscle Lands cycle ameliorates weakness induced by physical inactivity.

DOI：
10.1002/jcsm.13406
发表时间：
2024-02
期刊：
Journal of cachexia, sarcopenia and muscle
影响因子：
0
作者：
通讯作者：

Discovery of the gut microbial enzyme responsible for bilirubin reduction to urobilinogen.

发现负责将胆红素还原为尿胆原的肠道微生物酶。

DOI：
10.1101/2023.02.07.527579
发表时间：
2023
期刊：
bioRxiv : the preprint server for biology
影响因子：
0
作者：
Hall,Brantley;Levy,Sophia;Dufault-Thompson,Keith;Ndjite,GloryMinabou;Weiss,Ashley;Braccia,Domenick;Jenkins,Conor;Yang,Yiyan;Arp,Gabi;Abeysinghe,Stephenie;Jermain,Madison;Wu,ChihHao;Jiang,Xiaofang
通讯作者：
Jiang,Xiaofang

New Avenues of Heme Synthesis Regulation.