Spanaki Cleanthe, Kotzamani Dimitra, Plaitakis Andreas
Department of Neurology, Faculty of Medicine, School of Health Sciences, University of Crete, Heraklion, Crete, 71003, Greece.
Icahn School of Medicine at Mount Sinai, OneGustave L. Levy Place, New York, 10029, USA.
Neurochem Res. 2017 Jan;42(1):92-107. doi: 10.1007/s11064-016-1986-x. Epub 2016 Jul 16.
Mammalian glutamate dehydrogenase1 (GDH1) (E.C. 1.4.1.3) is a mitochondrial enzyme that catalyzes the reversible oxidative deamination of glutamate to α-ketoglutarate and ammonia while reducing NAD+ and/or NADP+ to NADH and/or NADPH. It links amino acid with carbohydrate metabolism, contributing to Krebs cycle anaplerosis, energy production, ammonia handling and redox homeostasis. Although GDH1 was one of the first major metabolic enzymes to be studied decades ago, its role in cell biology is still incompletely understood. There is however growing interest in a novel GDH2 isoenzyme that emerged via duplication in primates and underwent rapid evolutionary selection concomitant with prefrontal human cortex expansion. Also, the anaplerotic function of GDH1 and GDH2 is currently under sharp focus as this relates to the biology of glial tumors and other neoplasias. Here we used antibodies specific for human GDH1 (hGDH1) and human GDH2 (hGDH2) to study the expression of these isoenzymes in human tissues. Results revealed that both hGDH1 and hGDH2 are expressed in human brain, kidney, testis and steroidogenic organs. However, distinct hGDH1 and hGDH2 expression patterns emerged. Thus, while the Sertoli cells of human testis were strongly positive for hGDH2, they were negative for hGDH1. Conversely, hGDH1 showed very high levels of expression in human liver, but hepatocytes were virtually devoid of hGDH2. In human adrenals, both hGDHs were densely expressed in steroid-producing cells, with hGDH2 expression pattern matching that of the cholesterol side chain cleavage system involved in steroid synthesis. Similarly in human ovaries and placenta, both hGDH1 and hGDH2 were densely expressed in estrogen producing cells. In addition, hGDH1, being a housekeeping enzyme, was also expressed in cells that lack endocrine function. Regarding human brain, study of cortical sections using immunofluorescence (IF) with confocal microscopy revealed that hGDH1 and hGDH2 were both expressed in the cytoplasm of gray and white matter astrocytes within coarse structures resembling mitochondria. Additionally, hGDH1 localized to the nuclear membrane of a subpopulation of astrocytes and of the vast majority of oligodendrocytes and their precursors. Remarkably, hGDH2-specific staining was detected in human cortical neurons, with different expression patterns having emerged. One pattern, observed in large cortical neurons (some with pyramidal morphology), was a hGDH2-specific labeling of cytoplasmic structures resembling mitochondria. These were distributed either in the cell body-axon or on the cell surface in close proximity to astrocytic end-feet that encircle glutamatergic synapses. Another pattern was observed in small cortical neurons with round dense nuclei in which the hGDH2-specific staining was found in the nuclear membrane. A detailed description of these observations and their functional implications, suggesting that the GDH flux is used by different cells to serve some of their unique functions, is presented below.
哺乳动物谷氨酸脱氢酶1(GDH1)(E.C. 1.4.1.3)是一种线粒体酶,催化谷氨酸可逆氧化脱氨生成α-酮戊二酸和氨,同时将NAD⁺和/或NADP⁺还原为NADH和/或NADPH。它将氨基酸代谢与碳水化合物代谢联系起来,有助于三羧酸循环的回补反应、能量产生、氨的处理和氧化还原稳态。尽管GDH1是几十年前最早被研究的主要代谢酶之一,但其在细胞生物学中的作用仍未完全明确。然而,人们对一种新的GDH2同工酶的兴趣与日俱增,它是通过灵长类动物的基因复制产生的,并伴随着人类前额叶皮质的扩张经历了快速的进化选择。此外,GDH1和GDH2的回补功能目前成为研究热点,因为这与神经胶质瘤和其他肿瘤的生物学特性相关。在这里,我们使用针对人GDH1(hGDH1)和人GDH2(hGDH2)的特异性抗体研究了这些同工酶在人体组织中的表达。结果显示,hGDH1和hGDH2均在人脑、肾、睾丸和类固醇生成器官中表达。然而,出现了不同的hGDH1和hGDH2表达模式。因此,人睾丸的支持细胞对hGDH2呈强阳性,而对hGDH1呈阴性。相反,hGDH1在人肝脏中表达水平非常高,但肝细胞中几乎没有hGDH2。在人肾上腺中,两种hGDH在类固醇生成细胞中密集表达,hGDH2的表达模式与参与类固醇合成的胆固醇侧链裂解系统的表达模式相匹配。同样,在人卵巢和胎盘中,hGDH1和hGDH2均在雌激素产生细胞中密集表达。此外,hGDH1作为一种管家酶,也在缺乏内分泌功能的细胞中表达。关于人脑,使用免疫荧光(IF)结合共聚焦显微镜对皮质切片进行的研究表明,hGDH1和hGDH2均在类似于线粒体的粗大结构中的灰质和白质星形胶质细胞的细胞质中表达。此外,hGDH1定位于星形胶质细胞亚群以及绝大多数少突胶质细胞及其前体细胞的核膜上。值得注意的是,在人皮质神经元中检测到hGDH2特异性染色,出现了不同的表达模式。一种模式在大型皮质神经元(一些具有锥体形态)中观察到,是hGDH2对类似于线粒体的细胞质结构的特异性标记。这些结构分布在细胞体 - 轴突中或细胞表面,紧邻环绕谷氨酸能突触的星形胶质细胞终足。另一种模式在具有圆形致密核的小型皮质神经元中观察到,其中在核膜中发现了hGDH2特异性染色。以下是对这些观察结果及其功能意义的详细描述,表明不同细胞利用GDH通量来发挥其一些独特功能。