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首页> 外文期刊>Amino acids >Manifold decrease of sialic acid synthase in fetal Down syndrome brain
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Manifold decrease of sialic acid synthase in fetal Down syndrome brain

机译：胎儿唐氏综合症大脑中唾液酸合酶的歧管减少

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Background: Down syndrome (DS,trisomy 21) is the most common genetic cause of mental retardation.A large series of biochemical defects have been observed in fetal and adult DS brain that help in unraveling the molecular mechanisms underlying mental retardation.Aims: As sialylation of glycoconjugates plays an important role in brain development,this study aimed to look at the sialic acid metabolism by measuring sialic acid synthase (SAS; N-acetylneuraminate synthase) in early second trimester fetal control and DS brain.Results: In this regard,protein profiling was performed by two-dimensional gel electrophoresis coupled to matrix-assisted laser desorption/ ionization mass-spectrometry followed by database search and subsequent quantification of spot using specific software.SAS,the enzyme catalyzing synthesis of N-acetyl-neuraminic acid (syn: sialic acid) was represented as a single spot and found to be significantly and manifold reduced (P<0.01) in cortex of fetuses with DS (control vs.DS,0.052 +- 0.025 vs. 0.012 +- 0.006).Conclusion: The intriguing finding of the manifold decrease of SAS in DS fetal cerebral cortex as early as in the second trimester of pregnancy may help to explain the brain deficit observed in DS.Decreased SAS may well lead to altered sialic acid metabolism,required for brain development and,more specifically,for sialylation of key brain proteins,including neuronal cell adhesion molecule and myelin associated glycoprotein.
机译：背景：唐氏综合症（DS，21三体性疾病）是智力低下的最常见遗传原因。在胎儿和成人DS脑中观察到一系列生化缺陷，有助于揭示智力低下的分子机制。目的：唾液酸化糖缀合物的合成在大脑发育中起着重要作用，本研究旨在通过测定妊娠中期和DS大脑早期的唾液酸合酶（SAS; N-乙酰神经氨酸合酶）来研究唾液酸的代谢。结果：在这方面，蛋白质通过二维凝胶电泳与基质辅助激光解吸/电离质谱联用，进行数据库分析，然后使用特定软件对斑点进行定量分析.SAS，酶催化合成N-乙酰-神经氨酸（syn：唾液酸）被表示为一个单点，并发现在具有DS的胎儿皮质中显着且歧管减少（P <0.01）（对照vs. DS，0.052±0.025 vs. 0.012±0.006）。结论：早在妊娠中期，DS胎儿大脑皮层SAS大量减少的有趣发现可能有助于解释DS中观察到的脑缺陷。 SAS降低很可能导致唾液酸代谢改变，这是大脑发育所需要的，更具体地说，是唾液酸化关键大脑蛋白质（包括神经元细胞粘附分子和髓磷脂相关糖蛋白）所需的唾液酸。

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