Fédération des Maladies du Système Nerveux, APHP, Centre de référence maladies rares SLA, Hôpital Pitié-salpêtrière, Paris, France.
PLoS One. 2012;7(9):e45034. doi: 10.1371/journal.pone.0045034. Epub 2012 Sep 14.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder caused by the loss of motor neurons. Its etiology remains unknown, but several hypothesis have been raised to explain motor neuron death, including oxidative stress. Dysregulation of cellular iron metabolism can lead to increased oxidative stress, and existing data argue for a role of iron metabolism in ALS pathophysiology.
We performed a retrospective analysis of iron metabolism (IM) variables (serum levels of iron, transferrin, ferritin, and TSC for Transferrin Saturation Coefficient) in a cohort of 694 ALS patients and 297 healthy controls.
Serum ferritin levels and TSC were higher, whereas serum transferrin levels were lower in ALS patients than controls. In addition, patients with a high level serum ferritin had a shorter survival time compared to those with low level serum ferritin (618 days versus 921 days for men subgroup; p = .007). Site of onset and ALS-FRS score were not associated with IM variables.
This study suggests that ALS patients may have increased iron storage, as measured by increased serum ferritin and TSC. Elevated serum ferritin may also have a deleterious impact on survival in ALS.
肌萎缩侧索硬化症(ALS)是一种由运动神经元丧失引起的神经退行性疾病。其病因仍不清楚,但提出了几种假说来解释运动神经元死亡,包括氧化应激。细胞内铁代谢的失调可导致氧化应激增加,并且现有数据表明铁代谢在 ALS 病理生理学中起作用。
我们对 694 名 ALS 患者和 297 名健康对照者的铁代谢(IM)变量(血清铁、转铁蛋白、铁蛋白和转铁蛋白饱和度系数 TSC)进行了回顾性分析。
与对照组相比,血清铁蛋白水平和 TSC 较高,而转铁蛋白水平较低。此外,与血清铁蛋白水平较低的患者相比,血清铁蛋白水平较高的患者的存活时间较短(男性亚组的 618 天与 921 天;p=0.007)。发病部位和 ALS-FRS 评分与 IM 变量无关。
本研究表明,ALS 患者可能有铁储存增加,表现为血清铁蛋白和 TSC 增加。血清铁蛋白升高也可能对 ALS 患者的生存产生有害影响。