Targeted expression of genes coding for proteins specific to astrocytes, oligodendrocytes and myelin was performed in frontal cortex area 8 of Creutzfeldt-Jakob disease methionine/methionine and valine/valine (CJD MM1 and VV2, respectively) compared with controls. GFAP (glial fibrillary acidic protein) mRNA was up-regulated whereas SLC1A2 (solute carrier family 1 member 2, coding for glutamate transporter 1: GLT1), AQ4 (aquaporin 4), MPC1 (mitochondrial pyruvate carrier 1) and UCP5 (mitochondrial uncoupled protein 5) mRNAs were significantly down-regulated in CJD MM1 and CJD VV2, and GJA1 (connexin 43) in CJD VV2. OLIG1 and OLIG2 (oligodendocyte transcription factor 1 and 2, respectively), SOX10 (SRY-Box10) and oligodendroglial precursor cell (OPC) marker NG2 (neuronal/glial antigen) 2 were preserved, but GALC (coding for galactosylceramidase), SLC2A1 (solute carrier family 2 member 1: glucose transporter member 1: GLUT1) and MCT1 (monocarboxylic acid transporter 1) mRNA expression levels were significantly reduced in CJD MM1 and CJD VV2. Expression levels of most genes linked to myelin were not altered in the cerebral cortex in CJD. Immunohistochemistry to selected proteins disclosed individual variations but GFAP, Olig-2, AQ4 and GLUT1 correlated with mRNA levels, whereas GLT1 was subjected to individual variations. However, MPC1, UCP5 and MCT1 decrease was more closely related to the respective reduced neuronal immunostaining. These observations support the idea that molecular deficits linked to energy metabolism and solute transport in astrocytes and oligodendrocytes, in addition to neurons, are relevant in the pathogenesis of cortical lesions in CJD.

与对照组相比，在Creutzfeldt-Jakob病蛋氨酸/蛋氨酸和缬氨酸/缬氨酸（分别为CJD MM1和VV2）的额叶皮层区域8中进行了针对星形胶质细胞，少突胶质细胞和髓鞘特异性蛋白编码基因的靶向表达。GFAP（胶质纤维酸性蛋白）mRNA上调，而SLC1A2（溶质载体家族1成员2，编码谷氨酸转运蛋白1：GLT1），AQ4（水通道蛋白4），MPC1（线粒体丙酮酸载体1）和UCP5（线粒体非偶联蛋白） 5）CJD MM1和CJD VV2中的mRNA显着下调，CJD VV2中的GJA1（连接蛋白43）显着下调。OLIG1和OLIG2（分别是少突胶质细胞转录因子1和2），SOX10（SRY-Box10）和少突胶质前体细胞（OPC）标记NG2（神经元/神经胶质抗原）2，但保留了GALC（编码半乳糖苷神经酰胺酶），SLC2A1（溶质载体家族2）成员1：葡萄糖转运蛋白成员1：GLUT1）和MCT1（单羧酸转运蛋白1）在CJD MM1和CJD VV2中mRNA表达水平显着降低。CJD大脑皮层中大多数与髓磷脂相关的基因的表达水平没有改变。所选蛋白质的免疫组织化学显示个体差异，但GFAP，Olig-2，AQ4和GLUT1与mRNA水平相关，而GLT1则具有个体差异。然而，MPC1，UCP5和MCT1的减少与各自减少的神经元免疫染色更密切相关。这些观察结果支持这样的观点，即除了神经元外，与星形胶质细胞和少突胶质细胞中能量代谢和溶质运输有关的分子缺陷与CJD皮质病变的发病机制有关。