Lucas Elizabeth K, Dougherty Sarah E, McMeekin Laura J, Reid Courtney S, Dobrunz Lynn E, West Andrew B, Hablitz John J, Cowell Rita M
Departments of Psychiatry and Behavioral Neurobiology.
Neurobiology, and.
J Neurosci. 2014 Oct 22;34(43):14375-87. doi: 10.1523/JNEUROSCI.1222-14.2014.
Accumulating evidence strongly implicates the transcriptional coactivator peroxisome proliferator-activated receptor γ coactivator 1α (PGC-1α) in the pathophysiology of multiple neurological disorders, but the downstream gene targets of PGC-1α in the brain have remained enigmatic. Previous data demonstrate that PGC-1α is primarily concentrated in inhibitory neurons and that PGC-1α is required for the expression of the interneuron-specific Ca(2+)-binding protein parvalbumin (PV) throughout the cortex. To identify other possible transcriptional targets of PGC-1α in neural tissue, we conducted a microarray on neuroblastoma cells overexpressing PGC-1α, mined results for genes with physiological relevance to interneurons, and measured cortical gene and protein expression of these genes in mice with underexpression and overexpression of PGC-1α. We observed bidirectional regulation of novel PGC-1α-dependent transcripts spanning synaptic [synaptotagmin 2 (Syt2) and complexin 1 (Cplx1)], structural [neurofilament heavy chain (Nefh)], and metabolic [neutral cholesterol ester hydrolase 1 (Nceh1), adenylate kinase 1 (Ak1), inositol polyphosphate 5-phosphatase J (Inpp5j), ATP synthase mitochondrial F1 complex O subunit (Atp5o), phytanol-CoA-2hydroxylase (Phyh), and ATP synthase mitrochondrial F1 complex α subunit 1 (Atp5a1)] functions. The neuron-specific genes Syt2, Cplx1, and Nefh were developmentally upregulated in an expression pattern consistent with that of PGC-1α and were expressed in cortical interneurons. Conditional deletion of PGC-1α in PV-positive neurons significantly decreased cortical transcript expression of these genes, promoted asynchronous GABA release, and impaired long-term memory. Collectively, these data demonstrate that PGC-1α is required for normal PV-positive interneuron function and that loss of PGC-1α in this interneuron subpopulation could contribute to cortical dysfunction in disease states.
越来越多的证据有力地表明,转录共激活因子过氧化物酶体增殖物激活受体γ共激活因子1α(PGC-1α)与多种神经系统疾病的病理生理学有关,但PGC-1α在大脑中的下游基因靶点一直不明确。先前的数据表明,PGC-1α主要集中在抑制性神经元中,并且在整个皮质中,中间神经元特异性钙结合蛋白小白蛋白(PV)的表达需要PGC-1α。为了确定PGC-1α在神经组织中的其他可能转录靶点,我们对过表达PGC-1α的神经母细胞瘤细胞进行了微阵列分析,挖掘与中间神经元具有生理相关性的基因结果,并在PGC-1α表达不足和过表达的小鼠中测量这些基因的皮质基因和蛋白表达。我们观察到了新的PGC-1α依赖性转录本的双向调控,这些转录本涉及突触[突触结合蛋白2(Syt2)和复合体蛋白1(Cplx1)]、结构[神经丝重链(Nefh)]以及代谢[中性胆固醇酯水解酶1(Nceh1)、腺苷酸激酶1(Ak1)、肌醇多磷酸5-磷酸酶J(Inpp5j)、ATP合酶线粒体F1复合体O亚基(Atp5o)、植烷酰辅酶A-2-羟化酶(Phyh)以及ATP合酶线粒体F1复合体α亚基1(Atp5a1)]功能。神经元特异性基因Syt2、Cplx1和Nefh在发育过程中上调,其表达模式与PGC-1α一致,并在皮质中间神经元中表达。在PV阳性神经元中条件性删除PGC-1α会显著降低这些基因的皮质转录本表达,促进GABA的异步释放,并损害长期记忆。总体而言,这些数据表明正常的PV阳性中间神经元功能需要PGC-1α,并且该中间神经元亚群中PGC-1α的缺失可能导致疾病状态下的皮质功能障碍。