MITOCHONDRIAL FUNCTION IN CYSTINOSIS MYOPATHY

胱氨酸病肌病中的线粒体功能

• 批准号: 7374194
• 负责人: DORIS A TRAUNER
• 金额: $ 1.16万
• 依托单位: UNIVERSITY OF CALIFORNIA, SAN DIEGO
• 依托单位国家: 美国
• 财政年份: 2006
• 资助国家: 美国
• 起止时间: 2006-04-01 至 2006-11-30
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/7374194
• 关键词: biotin; carnitine; cofactor; riboflavin; thiamine

This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Determine whether the myopathy associated with nephropathic cystinosis is accompanied by mitochondrial dysfunction. Determine if daily supplements of carnitine, Coenzyme Q10, biotin, riboflavin and thiamine have a beneficial effect on the myopathy in these patients, perhaps improved mitochondrial function.

该子项目是利用NIH/NCRR资助的中心赠款提供的资源的许多研究子项目之一。子项目和研究者（PI）可能从另一个NIH来源获得主要资金，因此可以在其他CRISP条目中表示。所列机构为中心，不一定是研究者所在机构。确定肾病性胱氨酸病相关的肌病是否伴有线粒体功能障碍。确定是否每日补充肉毒碱，辅酶Q10，生物素，核黄素和硫胺素对这些患者的肌病有有益的影响，可能改善线粒体功能。