Odetti P, Angelini G, Dapino D, Zaccheo D, Garibaldi S, Dagna-Bricarelli F, Piombo G, Perry G, Smith M, Traverso N, Tabaton M
Department of Internal Medicine (DIMI), University of Genova, Italy.
Biochem Biophys Res Commun. 1998 Feb 24;243(3):849-51. doi: 10.1006/bbrc.1998.8186.
In Down's syndrome, the presence of three copies of chromosome 21 is associated with premature aging and progressive mental retardation sharing the pathological features of Alzheimer disease. Early cortical dysgenesis and late neuronal degeneration are probably caused by an overproduction of amyloid beta-peptide, followed by an increased cellular oxidation. Interestingly, chromosome 21 codes for superoxide-dismutase and amyloid beta precursor resulting, in Down's syndrome, in an overflow of these gene products and metabolites. We studied Down's fetal brain cortex to evaluate the presence and amount of lipid and protein oxidation markers; moreover, we quantified two forms of glycation end products that are known to be involved in the process of cellular oxidation. All these parameters are significantly increased in Down's fetal brains in comparison to controls, providing the evidence that accelerated brain glycoxidation occurs very early in the life of Down's syndrome subjects.
在唐氏综合征中，21号染色体三体的存在与过早衰老和进行性智力迟钝有关，这些症状具有阿尔茨海默病的病理特征。早期皮质发育异常和晚期神经元变性可能是由β-淀粉样肽过度产生，随后细胞氧化增加所致。有趣的是，21号染色体编码超氧化物歧化酶和淀粉样β前体，在唐氏综合征中，这些基因产物和代谢产物会出现溢出。我们研究了唐氏胎儿脑皮质，以评估脂质和蛋白质氧化标志物的存在及含量；此外，我们还对已知参与细胞氧化过程的两种糖基化终产物形式进行了定量。与对照组相比，所有这些参数在唐氏胎儿脑中均显著增加，这证明在唐氏综合征患者生命的早期就发生了加速的脑糖氧化。
