HS1-associated protein X-1 (HAX-1) and presenilin-associated rhomboid-like protein (PALR) were reported to play an important role in the activation of HtrA2/Omi, which is also designated PARK13, in the mitochondria. To elucidate the role of HAX-1 and PARL in patients with Parkinson's disease (PD) and dementia with Lewy bodies (DLB), we performed immunohistochemical studies on HtrA2/Omi, HAX-1 and PARL using autopsied brains from 8 normal subjects, 10 patients with PD and 5 patients with DLB. In accordance with our previous report, brainstem-type and cortical Lewy bodies were strongly immunopositive for HtrA2/Omi. In the normal brains, HAX-1 and PARL immunoreactivities were observed in various types of neurons in the cerebral cortex, midbrain, and upper pons. HAX-1 and PARL immunoreactivities were also observed in the remaining neurons, and brainstem-type and cortical Lewy bodies were intensely immunoreactive for HAX-1 and PARL. Both immunoreactivities were localized to the halo or core of brainstem-type Lewy bodies. Our results suggest that brainstem-type and cortical Lewy bodies may contain HAX-1 and PARL as well as HtrA2/Omi, and that these proteins may partially contribute to the formation of Lewy bodies and may be associated with the pathogenesis of PD and DLB.

中文翻译：

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HAX-1 和 PARL 在帕金森病和路易体痴呆中脑干型和皮质型路易体的积累

据报道，HS1 相关蛋白 X-1 (HAX-1) 和早老素相关菱形样蛋白 (PALR) 在线粒体中 HtrA2/Omi（也称为 PARK13）的激活中起重要作用。为了阐明 HAX-1 和 PARL 在帕金森病 (PD) 和路易体痴呆 (DLB) 患者中的作用，我们使用来自 8 名正常受试者、10 PD 患者和 DLB 患者 5 例。根据我们之前的报告，脑干型和皮质路易体对 HtrA2/Omi 呈强免疫阳性。在正常大脑中，在大脑皮层、中脑和上脑桥的各种类型的神经元中观察到HAX-1和PARL免疫反应性。在剩余的神经元中也观察到了 HAX-1 和 PARL 免疫反应性，脑干型和皮质路易体对 HAX-1 和 PARL 具有强烈的免疫反应性。两种免疫反应都定位于脑干型路易体的晕圈或核心。我们的研究结果表明，脑干型和皮质路易体可能含有HAX-1和PARL以及HtrA2/Omi，这些蛋白质可能部分促成了路易体的形成，并可能与PD和DLB的发病机制有关。