Department of Oral Medicine, School of Dental Medicine, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
Department of Neurology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
J Neurochem. 2023 Jun;165(5):722-740. doi: 10.1111/jnc.15773. Epub 2023 Feb 16.
White matter deficits are a common neuropathologic finding in neurologic disorders, including HIV-associated neurocognitive disorders (HAND). In HAND, the persistence of white matter alterations despite suppressive antiretroviral (ARV) therapy suggests that ARVs may be directly contributing to these impairments. Here, we report that a frontline ARV, bictegravir (BIC), significantly attenuates remyelination following cuprizone-mediated demyelination, a model that recapitulates acute demyelination, but has no impact on already formed mature myelin. Mechanistic studies utilizing primary rat oligodendrocyte precursor cells (OPCs) revealed that treatment with BIC leads to significant decrease in mature oligodendrocytes accompanied by lysosomal deacidification and impairment of lysosomal degradative capacity with no alterations in lysosomal membrane permeability or total lysosome number. Activation of the endolysosomal cation channel TRPML1 prevents both lysosomal deacidification and impairment of oligodendrocyte differentiation by BIC. Lastly, we show that deacidification of lysosomes by compounds that raise lysosomal pH is sufficient to prevent maturation of oligodendrocytes. Overall, this study has uncovered a critical role for lysosomal acidification in modulating oligodendrocyte function and has implications for neurologic diseases characterized by lysosomal dysfunction and white matter abnormalities.
脑白质病变是神经紊乱的一种常见神经病理学表现,包括与 HIV 相关的神经认知障碍(HAND)。在 HAND 中,尽管抗逆转录病毒(ARV)治疗具有抑制作用,但仍存在脑白质改变,这表明 ARV 可能直接导致这些损伤。在这里,我们报告称,一线 ARV 药物比替拉韦(BIC)显著抑制了由杯状醇诱导的脱髓鞘后再髓鞘形成,该模型再现了急性脱髓鞘,但对已形成的成熟髓鞘没有影响。利用原代大鼠少突胶质前体细胞(OPC)进行的机制研究表明,BIC 治疗导致成熟少突胶质细胞数量显著减少,同时伴有溶酶体去酸化和溶酶体降解能力受损,而溶酶体膜通透性或总溶酶体数量没有改变。内溶酶体阳离子通道 TRPML1 的激活可防止 BIC 引起的溶酶体去酸化和少突胶质细胞分化受损。最后,我们表明,通过提高溶酶体 pH 值的化合物使溶酶体去酸化足以防止少突胶质细胞成熟。总的来说,这项研究揭示了溶酶体酸化在调节少突胶质细胞功能中的关键作用,这对以溶酶体功能障碍和脑白质异常为特征的神经疾病具有重要意义。