Danuta Maślinska, Department of Clinical and Experimental Neuropathology, Mossakowski Medical Research Centre, PASci, Pawinskiego 5, 02-106 Warszawa, e-mail:
Folia Neuropathol. 2013;51(4):312-8. doi: 10.5114/fn.2013.39720.
Zinc-alpha-2-glycoprotein (ZAG) is a protein identified as a lipid-mobilizing factor participating in a lipid catabolism. In spite of intensive studies conducted during last five decades, the role of this protein in processes of neurodegeneration remains unclear. The aim of our study was to examine the presence of ZAG protein in the brain of patients with Krabbe's disease, which is considered as a psychosine lipidosis caused by a mutation of a known gene. We found intracellular and extracellular localization of ZAG in the brain of Krabbe's disease patients but in the brain of control age-matched patients, ZAG was not detected. Distribution of ZAG in the brain suggests that the influx of ZAG into the brain involved a blood-brain barrier mechanism and adenoreceptors localized on astrocytes and some neurons.
锌-α-2-糖蛋白（ZAG）是一种被确定为参与脂质分解代谢的脂动员因子。尽管在过去五十年中进行了大量研究，但这种蛋白质在神经退行性变过程中的作用仍不清楚。我们的研究目的是检查 Krabbe 病患者大脑中 ZAG 蛋白的存在，Krabbe 病被认为是一种由已知基因突变引起的神经鞘脂贮积症。我们发现 ZAG 在 Krabbe 病患者的大脑中有细胞内和细胞外定位，但在年龄匹配的对照组患者的大脑中没有检测到 ZAG。ZAG 在大脑中的分布表明，ZAG 流入大脑涉及血脑屏障机制和定位于星形胶质细胞和某些神经元上的肾上腺素能受体。
