Wallimann T, Hemmer W
Institute for Cell Biology, Swiss Federal Institute of Technology, ETH-Hönggerberg, Zürich.
Mol Cell Biochem. 1994 Apr-May;133-134:193-220. doi: 10.1007/BF01267955.
Over the past years, a concept for creatine kinase function, the 'PCr-circuit' model, has evolved. Based on this concept, multiple functions for the CK/PCr-system have been proposed, such as an energy buffering function, regulatory functions, as well as an energy transport function, mostly based on studies with muscle. While the temporal energy buffering and metabolic regulatory roles of CK are widely accepted, the spatial buffering or energy transport function, that is, the shuttling of PCr and Cr between sites of energy utilization and energy demand, is still being debated. There is, however, much circumstantial evidence, that supports the latter role of CK including the distinct, isoenzyme-specific subcellular localization of CK isoenzymes, the isolation and characterization of functionally coupled in vitro microcompartments of CK with a variety of cellular ATPases, and the observed functional coupling of mitochondrial oxidative phosphorylation with mitochondrial CK. New insight concerning the functions of the CK/PCr-system has been gained from recent M-CK null-mutant transgenic mice and by the investigation of CK localization and function in certain highly specialized non-muscle tissues and cells, such as electrocytes, retina photoreceptor cells, brain cells, kidney, salt glands, myometrium, placenta, pancreas, thymus, thyroid, intestinal brush-border epithelial cells, endothelial cells, cartilage and bone cells, macrophages, blood platelets, tumor and cancer cells. Studies with electric organ, including in vivo 31P-NMR, clearly reveal the buffer function of the CK/PCr-system in electrocytes and additionally corroborate a direct functional coupling of membrane-bound CK to the Na+/K(+)-ATPase. On the other hand, experiments with live sperm and recent in vivo 31P-NMR measurements on brain provide convincing evidence for the transport function of the CK/PCr-system. We report on new findings concerning the isoenzyme-specific cellular localization and subcellular compartmentation of CK isoenzymes in photoreceptor cells, in glial and neuronal cells of the cerebellum and in spermatozoa. Finally, the regulation of CK expression by hormones is discussed, and new developments concerning a connection of CK with malignancy and cancer are illuminated. Most interesting in this respect is the observed upregulation of CK expression by adenoviral oncogenes.
在过去几年中,肌酸激酶功能的一种概念,即“磷酸肌酸循环”模型已经形成。基于这一概念,人们提出了肌酸激酶/磷酸肌酸系统的多种功能,如能量缓冲功能、调节功能以及能量运输功能,这些大多是基于对肌肉的研究。虽然肌酸激酶的瞬时能量缓冲和代谢调节作用已被广泛接受,但空间缓冲或能量运输功能,即磷酸肌酸和肌酸在能量利用部位和能量需求部位之间的穿梭,仍存在争议。然而,有许多间接证据支持肌酸激酶的后一种作用,包括肌酸激酶同工酶独特的、同工酶特异性的亚细胞定位、肌酸激酶与多种细胞ATP酶功能偶联的体外微区室的分离和表征,以及观察到的线粒体氧化磷酸化与线粒体肌酸激酶的功能偶联。关于肌酸激酶/磷酸肌酸系统功能的新见解来自最近的M-CK基因敲除突变转基因小鼠,以及对某些高度特化的非肌肉组织和细胞(如电细胞、视网膜光感受器细胞、脑细胞、肾脏、盐腺、子宫肌层、胎盘、胰腺、胸腺、甲状腺、肠刷状缘上皮细胞、内皮细胞、软骨和骨细胞、巨噬细胞、血小板、肿瘤和癌细胞)中肌酸激酶定位和功能的研究。对电器官的研究,包括体内31P-NMR,清楚地揭示了肌酸激酶/磷酸肌酸系统在电细胞中的缓冲功能,并进一步证实了膜结合肌酸激酶与Na+/K(+)-ATP酶的直接功能偶联。另一方面,对活精子的实验和最近对大脑的体内31P-NMR测量为肌酸激酶/磷酸肌酸系统的运输功能提供了令人信服的证据。我们报告了关于光感受器细胞、小脑胶质细胞和神经元细胞以及精子中肌酸激酶同工酶的同工酶特异性细胞定位和亚细胞区室化的新发现。最后,讨论了激素对肌酸激酶表达的调节,并阐明了肌酸激酶与恶性肿瘤和癌症联系的新进展。在这方面最有趣的是观察到腺病毒癌基因对肌酸激酶表达的上调。