PATHOGENIC ROLE FOR A SERUM FACTOR IN REYE'S SYNDROME

雷氏综合征中血清因子的致病作用

• 批准号: 3395846
• 负责人: JUNE R APRILLE
• 金额: $ 6.04万
• 依托单位: TUFTS UNIVERSITY MEDFORD
• 依托单位国家: 美国
• 财政年份: 1979
• 资助国家: 美国
• 起止时间: 1979-01-01 至 1986-08-31
• 项目状态: 已结题
• 来源: https://reporter.nih.gov/project-details/3395846
• 关键词: Reye's syndrome; bioassay; bioenergetics; blood tests; cytochromes; diagnostic tests; disease /disorder proneness /risk; electron transport; fatty acid metabolism; fluorescence spectrometry; genetic disorder diagnosis; human tissue; inborn urea cycle disorder; liver cells; mass spectrometry; mitochondria; radiotracer; serum; tissue /cell culture; virus diseases

Reye's Syndrome is characterized by severe mitochondrial injury as determined by electron microscpoy of liver biopsies obtained from children in critical stages of illness. The goal of this project is to study possible causes of the mitochondrial damage. The study is based on the hypothesis that a substance deleterious to mitochondria might be detected in serum. Reye's patient serum will therefore be tested in several in vitro assays of mitochondrial function, including: citrulline synthesis, oxidative phosphorylation, fatty acid oxidation, and protein synthesis. If Reye's serum has effects different from those of normal serum, the active substance will be isolated and identified to determine a possible pathogenic role in this illness. In addition, the study of how normal mitochondrial function is regulated will continue in order to increase the potential for predicting the common denominator for the diverse metabolic abnormalities that characterize Reye's syndrome.

雷氏综合症的特征是严重的线粒体损伤，如下所示： 关键阶段儿童肝活检的电子显微镜检查 的疾病。 该项目的目标是研究造成这种情况的可能原因 线粒体损伤。 该研究基于以下假设：一种物质 血清中可能检测到对线粒体有害的物质。 雷氏病人血清 因此将在线粒体功能的几种体外测定中进行测试， 包括：瓜氨酸合成、氧化磷酸化、脂肪酸 氧化、蛋白质合成。 如果雷耶血清的效果与 正常血清中的活性物质将被分离和鉴定 确定这种疾病中可能的致病作用。 此外，研究 正常线粒体功能的调节方式将继续下去，以便 增加预测多样化的共同点的潜力 雷氏综合征特征的代谢异常。

项目成果

Net uptake of adenine nucleotides in isolated rat liver mitochondria.

离体大鼠肝线粒体中腺嘌呤核苷酸的净摄取。

• DOI: 10.1016/0014-5793(80)80934-3
• 发表时间: 1980
• 期刊: FEBS letters
• 影响因子: 3.5
• 作者: Asimakis,GK;Aprille,JR
• 通讯作者: Aprille,JR

Acute postnatal regulation of pyruvate carboxylase activity by compartmentation of mitochondrial adenine nucleotides.

通过线粒体腺嘌呤核苷酸的区室对丙酮酸羧化酶活性进行急性产后调节。

• DOI: 10.1016/0304-4165(81)90080-5
• 发表时间: 1981
• 期刊: Biochimica et biophysica acta
• 作者: Aprille,JR;Yaswen,P;Rulfs,J
• 通讯作者: Rulfs,J

Adenine nucleotide pool size, adenine nucleotide translocase activity, and respiratory activity in newborn rabbit liver mitochondria.

新生兔肝线粒体中的腺嘌呤核苷酸池大小、腺嘌呤核苷酸转位酶活性和呼吸活性。

• DOI: 10.1016/0005-2728(82)90035-4
• 发表时间: 1982
• 期刊: Biochimica et biophysica acta
• 作者: Rulfs,J;Aprille,JR
• 通讯作者: Aprille,JR

Adenine nucleotide content of liver mitochondria increases after glucagon treatment of rats or isolated hepatocytes.

胰高血糖素处理大鼠或分离的肝细胞后，肝线粒体的腺嘌呤核苷酸含量增加。

• DOI: 10.1016/0006-291x(82)90905-6
• 发表时间: 1982
• 期刊: Biochemical and biophysical research communications
• 影响因子: 3.1
• 作者: Aprille,JR;Nosek,MT;BrennanJr,WA
• 通讯作者: BrennanJr,WA

Citrulline synthesis: regulation by alterations in the total mitochondrial adenine nucleotide content.

瓜氨酸合成：通过改变线粒体腺嘌呤核苷酸总量进行调节。

• DOI: 10.1016/0003-9861(82)90433-7
• 发表时间: 1982
• 期刊: Archives of biochemistry and biophysics
• 影响因子: 3.9
• 作者: Goldstein,FG;Aprille,JR
• 通讯作者: Aprille,JR