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HUMAN DISORDERS OF COPPER METABOLISM: RECENT ADVANCES AND MAIN CHALLENGES

人类铜代谢紊乱：最新进展和主要挑战

基本信息

批准号：
8459097
负责人：
SVETLANA LUTSENKO
金额：
$ 1.9万
依托单位：
JOHNS HOPKINS UNIVERSITY
依托单位国家：
美国
项目类别：
财政年份：
2013
资助国家：
美国
起止时间：
2013-04-02 至 2014-03-31
项目状态：
已结题

来源：
https://reporter.nih.gov/project-details/8459097
关键词：
Address Affect Animal Model Area Basic Science Beryllium Biological Biological Markers Brain Cells Chelating Agents Child Clinical Clinical Research Clinical Sciences Collaborations Communities Copper Development Diagnosis Diagnostic Disease Disease Association Educational workshop Etiology Gene Transfer Generations Genetic Goals Growth and Development function Health Hearing Heart Hypertrophy Hepatolenticular Degeneration Homeostasis Human Human Pathology Human body Image Immune response Impairment Kidney Knowledge Laboratories Learning Link Liver Longevity Measurement Mediating Menkes Kinky Hair Syndrome Messenger RNA Metabolic Metabolic Diseases Metabolism Methodology Molecular Monitor Neuraxis Neurologic Organ Organism Outcome Participant Pathology Patient Representative Patients Physicians Physiological Processes Positron-Emission Tomography Recommendation Research Resolution Scientist Targeted Research Technology Tissues Training Programs Translating Translational Research Translations Update Viral Work Writing angiogenesis base clinical practice disease diagnosis fundamental research heart function hypocupremia improved in vivo innovation interest liver function meetings novel strategies public health relevance research and development sensor translational study trend

项目摘要

DESCRIPTION (provided by applicant): Copper is an essential element that is necessary for the development of central nervous system, normal heart and liver functions, angiogenesis, immune response and many other physiologic processes. Copper deficiency is associated with cardiac hypertrophy, morphologic and functional pathologies in the brain, whereas excess copper disrupts organ function resulting in liver and kidney damage and neurologic impairment. Recent progress in disease diagnoses has significantly expanded the spectrum of disorders associated with abnormalities of copper metabolism. As a result, the need for beter mechanistic understanding of human copper metabolism in health and disease has greatly increased. This workshop would be the first meeting to specifically focus on the spectrum of human disorders associated with abnormalities of copper metabolism and the mechanisms behind copper-dependent pathologies. The issues related to diagnoses and treatment of copper-related disorders will be identified to guide mechanistic studies. Clinical, Basic Science and Patients perspective wil be presented at the meeting; the outcomes/conclusions of the workshop will be disseminated to all three groups.

描述（申请人提供）：铜是中枢神经系统发育、正常心脏和肝脏功能、血管生成、免疫反应和许多其他生理过程所必需的必需元素。铜缺乏与心脏肥大、大脑形态和功能病理有关，而过量的铜会破坏器官功能，导致肝肾损伤和神经系统损伤。疾病诊断的最新进展显着扩大了与铜代谢异常相关的疾病范围。因此，对健康和疾病中人体铜代谢更好的机制了解的需求大大增加。本次研讨会将是第一次专门关注与铜代谢异常相关的人类疾病范围以及铜依赖性病理背后的机制的会议。将确定与铜相关疾病的诊断和治疗相关的问题以指导机制研究。会议将介绍临床、基础科学和患者的观点；研讨会的成果/结论将分发给所有三个群体。