Department of Neurology, Teikyo University Chiba Medical Center, 3426–3 Anesaki, Ichihara, Japan.
J Neurol Sci. 2011 Oct 15;309(1-2):110-4. doi: 10.1016/j.jns.2011.07.003. Epub 2011 Jul 28.
It has been demonstrated that progranulin (PGRN) is a neurotrophic factor that enhances neuronal survival and axonal growth. Several lines of evidence have indicated that PGRN plays a role in the pathomechanism of amyotrophic lateral sclerosis (ALS). However, there has no study of PGRN in ALS skin. We made a quantitative immunohistochemical study of the expression of PGRN in the skin from 18 patients with sporadic ALS and 13 control subjects. Immunohistochemistry for PGRN demonstrated cytoplasmic activity in the epidermis and in some blood vessels and glands. Numerous PGRN-positive (PGRN+) cells were observed in the epidermis in ALS patients, which became more marked as ALS progressed. PGRN immunoreactivity of PGRN+cells was markedly positive in the epidermis in ALS patients. The proportion of PGRN+cells in the epidermis in ALS patients was significantly higher (p<0.001) than in controls. There was a significant positive relationship (r = 0.83, p<0.001) between the proportion and duration of illness in ALS patients. These data suggest that changes of PGRN in ALS skin are related to the disease process and that metabolic alteration of PGRN may take place in the skin of patients with ALS.
已有研究表明颗粒体蛋白前体（PGRN）是一种神经营养因子，可增强神经元的存活和轴突生长。有几行证据表明 PGRN 在肌萎缩侧索硬化症（ALS）的发病机制中发挥作用。然而，目前尚无关于 ALS 皮肤中 PGRN 的研究。我们对 18 例散发性 ALS 患者和 13 例对照者的皮肤中 PGRN 的表达进行了定量免疫组织化学研究。PGRN 的免疫组织化学显示表皮和一些血管和腺体中的细胞质活性。在 ALS 患者的表皮中观察到大量 PGRN 阳性（PGRN+）细胞，随着 ALS 的进展，这些细胞变得更加明显。ALS 患者表皮中 PGRN+细胞的 PGRN 免疫反应明显阳性。ALS 患者表皮中 PGRN+细胞的比例明显高于对照组（p<0.001）。ALS 患者表皮中 PGRN+细胞的比例与疾病持续时间之间存在显著的正相关关系（r = 0.83，p<0.001）。这些数据表明，ALS 皮肤中 PGRN 的变化与疾病过程有关，并且 PGRN 的代谢改变可能发生在 ALS 患者的皮肤中。
