Possible role of polyamines in gyrate atrophy.
Possible role of polyamines in gyrate atrophy.
复制标题
多胺在脑回萎缩中的可能作用。
DOI：
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发表时间：
2000
影响因子：
3.1
通讯作者：
R. Punitham
中科院分区：
文献类型：
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作者：
K. Sulochana;S. Ramakrishnan;L. Mahesh;R. Punitham
目的
回旋状萎缩（GA）的特点是高鸟氨酸血症和降低的鸟氨酸氨基转移酶（OAT）。然而，也有GA患者没有高鸟氨酸血症和那些高鸟氨酸血症没有GA。据报道，GA的一些病例具有低赖氨酸。本研究的目的是确定多胺、鸟氨酸的代谢物和赖氨酸是否在GA中具有任何诊断作用。
方法
通过二维纸色谱法估计血浆中的鸟氨酸，洗脱有色斑点，并使用分光光度计在560 nm处测量吸光度。淋巴细胞中的OAT测定是使用鸟氨酸作为底物通过荧光定量法进行的。血和尿中的多胺用正丁醇提取，苯甲酰化，并与HPLC分析;腐胺，精胺，亚精胺，和尸胺分别测定在254 nm的紫外检测器使用ODS，G18柱，63%甲醇作为溶剂。
结果
在调查的7例患者中，6例具有GA的典型特征。1例诊断为非典型视网膜色素变性（例3）。前5例患者的鸟氨酸升高，OAT降低，但第6和7例患者的鸟氨酸接近正常，第7例患者的OAT接近正常。然而，与正常人相比，所有7名患者的尿液中总多胺水平均升高。五个增加腐胺和三个增加精胺。7例尿尸胺均减少。因此，尽管存在与鸟氨酸和OAT不一致的情况，但所有7例患者的鸟氨酸多胺升高，尸胺降低。
结论
除了估计鸟氨酸和OAT在GA中，它建议，尿多胺可能进行分析，因为后者似乎更好地与临床状况相关，并在更大程度上帮助诊断。此外，虽然鸟氨酸是一种无害的氨基酸，但已知多胺会破坏DNA和蛋白质。
PURPOSE
Gyrate atrophy (GA) is marked by hyperornithinemia and lowered ornithine amino transferase (OAT). However there are patients of GA without hyperornithinemia and those with hyperornithinemia without GA. Some cases of GA have been reported to have low lysine. The purpose of the study was to determine if polyamines, the metabolites of ornithine, and lysine have any diagnostic role in GA.
METHODS
Ornithine in plasma was estimated by two-dimensional paper chromatography, with elution of the coloured spot, and the absorbance measured using a spectrophotometer at 560 nm. OAT assay in lymphocytes was done spectrophotometrically using ornithine as substrate. Blood and urinary polyamines were extracted with n-butanol, benzoylated and analysed with HPLC; putrescine, spermine, spermidine, and cadaverine were assayed individually at 254 nm with the UV detector using ODS, G18 column with 63% methanol as solvent.
RESULTS
Of the 7 patients investigated, 6 had features typical of GA. One was diagnosed to have atypical retinitis pigmentosa (case 3). The first five cases had elevated ornithine and diminished OAT, but cases 6 and 7 had near-normal ornithine and case 7 had near-normal OAT. However, all 7 patients had increased levels of total polyamines in urine compared to normals. Five had increased putrescine and three had increased spermine. All the 7 had decreased cadaverine in urine. Thus, though there were inconsistencies with ornithine and OAT, all the 7 patients had elevated polyamines from ornithine and decreased cadaverine.
CONCLUSION
In addition to estimating ornithine and OAT in GA, it is suggested that urinary polyamines may be analysed as the latter appears to correlate better with the clinical condition and help in the diagnosis to a greater extent. Moreover, while ornithine is an innocuous amino acid, polyamines are known to damage DNA and proteins.