Amyotrophic lateral sclerosis (ALS) is a progressive multifactorial disease characterized by the loss of motor neurons (MNs). Not all MNs undergo degeneration: neurons of the oculomotor nucleus, which regulate eye movements, are less vulnerable compared to hypoglossal nucleus MNs. Several molecular studies have been performed to understand the different vulnerability of these MNs. By analyzing postmortem samples from ALS patients to other unrelated decedents, the differential genomic pattern between the two nuclei has been profiled. Among identified genes, adenylate cyclase activating polypeptide 1 (ADCYAP1) gene, encoding for pituitary adenylate cyclase-activating polypeptide (PACAP), was found significantly up-regulated in the oculomotor versus hypoglossal nucleus suggesting that it could play a trophic effect on MNs in ALS. In the present review, some aspects regarding the different vulnerability of oculomotor and hypoglossal nucleus to degeneration will be summarized. The distribution and potential role of PACAP on these MNs as studied largely in an animal model of ALS compared to controls, will be discussed.

中文翻译：

---

ALS 期间动眼神经核与舌下神经核的差异易损性：PACAP 的参与

肌萎缩侧索硬化 (ALS) 是一种进行性多因素疾病，其特征是运动神经元 (MN) 的丧失。并非所有的 MN 都会发生变性：与舌下神经核 MN 相比，调节眼球运动的动眼神经核的神经元不那么脆弱。已经进行了几项分子研究来了解这些 MN 的不同脆弱性。通过分析从 ALS 患者到其他无关死者的死后样本，已经分析了两个细胞核之间的差异基因组模式。在已鉴定的基因中，编码垂体腺苷酸环化酶激活多肽（PACAP）的腺苷酸环化酶激活多肽 1（ADCYAP1）基因在动眼神经核与舌下神经核中显着上调，表明它可以对 ALS 中的 MN 发挥营养作用. 在本综述中，将总结有关动眼神经核和舌下神经核变性的不同脆弱性的一些方面。将讨论 PACAP 对这些 MN 的分布和潜在作用，主要是在 ALS 动物模型中与对照相比进行了研究。