Department of Biochemistry, Faculty of Pharmaceutical Sciences, Iwate Medical University.
Department of Biological Sciences, Institute of Scientific and Industrial Research, Osaka University.
Proc Jpn Acad Ser B Phys Biol Sci. 2019;95(6):261-277. doi: 10.2183/pjab.95.018.
Vacuolar-type ATPase (V-ATPase), initially identified in yeast and plant vacuoles, pumps protons into the lumen of organelles coupled with ATP hydrolysis. The mammalian counterpart is found ubiquitously in endomembrane organelles and the plasma membrane of specialized cells such as osteoclasts. V-ATPase is also present in unique organelles such as insulin secretory granules, neural synaptic vesicles, and acrosomes of spermatozoa. Consistent with its diverse physiological roles and unique localization, the seven subunits of V-ATPase have 2-4 isoforms that are organelle- or cell-specific. Subunits of the enzyme function in trafficking organelles and vesicles by interacting with small molecule GTPases. During osteoclast differentiation, one of the four isoforms of subunit a, a3, is indispensable for secretory lysosome trafficking to the plasma membrane. Diseases such as osteopetrosis, renal acidosis, and hearing loss are related to V-ATPase isoforms. In addition to its role as an enzyme, V-ATPase has versatile physiological roles in eukaryotic cells.
液泡型 ATP 酶(V-ATPase)最初在酵母和植物液泡中被发现,它通过与 ATP 水解偶联将质子泵入细胞器腔中。其在哺乳动物中广泛存在于内质网细胞器和破骨细胞等特殊细胞的质膜中。V-ATPase 也存在于独特的细胞器中,如胰岛素分泌颗粒、神经突触小泡和精子顶体。与其多样化的生理作用和独特的定位相一致,V-ATPase 的七个亚基有 2-4 种同工型,具有细胞器或细胞特异性。该酶的亚基通过与小分子 GTP 酶相互作用在细胞器和囊泡运输中发挥作用。在破骨细胞分化过程中,亚基 a 的四个同工型之一 a3 对于分泌溶酶体向质膜的运输是必不可少的。与 V-ATPase 同工型相关的疾病有骨硬化症、肾性酸中毒和听力损失等。除了作为一种酶的作用外,V-ATPase 在真核细胞中还具有多种生理作用。