Amyotrophic lateral sclerosis (ALS) is a common degenerative disease of the central nervous system concerning a progressive loss of upper and lower motor neurons. While 5%–10% of patients are diagnosed with the inherited form of the disease, the vast majority of patients suffer from the less characterized sporadic form of ALS (sALS). As the wobbler mouse and the ALS show striking similarities in view of phenotypical attributes, the mouse is rated as an animal model for the disease. Recent investigations show the importance of nicotinamide adenine dinucleotide (NAD⁺) and its producing enzyme nicotinic acid mononucleotide transferase 2 (Nmnat2) for neurodegeneration as well as for the preservation of health of the neuronal cells. Furthermore, it is newly determined that these molecules show significant downregulations in the spinal cord of wobbler mice in the stable phase of disease development. Here, we were able to prove a positive benefit on affected motor neurons from an additional NAD⁺ supply as well as an increase in the Nmnat2 level through caffeine treatment in cells in vitro. In addition, first assumptions about the importance of endogenous and exogenous factors that have an influence on the wellbeing of motor nerve cells in the model of ALS can be considered.

中文翻译：

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咖啡因和NAD +可以提高离体摇晃细胞的运动神经完整性。

肌萎缩性侧索硬化症（ALS）是中枢神经系统的常见退行性疾病，涉及上，下运动神经元的逐渐丧失。尽管有5％–10％的患者被诊断出患有遗传性疾病，但绝大多数患者患有特征较少的ALS（sALS）。考虑到表型属性，摆动鼠标和ALS显示出惊人的相似性，因此该鼠标被视为该疾病的动物模型。最近的研究表明烟酰胺腺嘌呤二核苷酸（NAD ⁺）及其产生的酶烟酸单核苷酸转移酶2（Nmnat2），用于神经退行性疾病以及保护神经元细胞的健康。此外，新近确定在疾病发展的稳定阶段，这些分子在摇摆小鼠的脊髓中显示出明显的下调。在这里，我们能够通过额外的NAD ⁺供应以及体外咖啡因处理对Nmnat2水平的提高，证明对受影响的运动神经元有积极的好处。此外，可以考虑有关在ALS模型中影响运动神经细胞健康的内源性和外源性因素的重要性的第一个假设。