Deuss Miriam, Reiss Karina, Hartmann Dieter
Dept. of Anatomy, Division of Neuroanatomy, University of Bonn, Nussallee 10, D-53115 Bonn, Federal Republic Germany.
Curr Alzheimer Res. 2008 Apr;5(2):187-201. doi: 10.2174/156720508783954686.
Disintegrin metalloproteases of the ADAM family form a large (at present > 40 members in mammals) family of multidomain membrane proteins that in their ectodomain combine a cystein-rich, disintegrin and a zinc metalloprotease domain. Via their metalloprotease domain, ADAMs are often implicated in ectodomain shedding, either to release e.g. growth factors or to initiate further intracellular signalling via regulated intramembrane proteolysis. Mainly based upon overexpression studies in vehicle cells, three of them, ADAMs 9, 10 and 17, have been proposed to act as alpha-secretases for amyloid precursor protein (APP). It is striking thereby that this role has since then remained somewhat ill-defined, as APP processing in ADAM9 deficient neurons is unaltered, and also ADAM10 deficient murine embryonic fibroblasts exhibit at best a highly variable reduction in alpha-secretase activity. However, during the past years, numerous other substrates have been linked to all three sheddases, the cleavage of which in some cases appears to be strikingly more important for the organism than APP processing. Most notably, the perinatally lethal phenotype of ADAM17 knockout mice is dominated by a loss of growth factor shedding, while the even earlier fatal effects of ADAM10 deficiency exhibit key features of disabled Notch signalling and possibly also cadherin processing defects. In this review, we will summarize the published data on the "non-APP" functions of all three ADAMs, the further evaluation of which may be crucial when attempting to treat Alzheimer s Disease by increasing their expression and/or activity. As the knockouts of ADAM10 and ADAM17 are only informative for their roles in (early) development, while a number of recently assigned new substrates play crucial roles in the normal and/or diseased adult organism as well, work on conditional knockout models will be crucial to fully characterize both the full functional portfolio of (candidate) alpha-secretases as well as their clinical relevance, which may go way beyond Alzheimer s Disease.
ADAM家族的解整合素金属蛋白酶构成了一个大型的(目前哺乳动物中有超过40个成员)多结构域膜蛋白家族,其胞外结构域结合了一个富含半胱氨酸的解整合素结构域和一个锌金属蛋白酶结构域。通过其金属蛋白酶结构域,ADAMs常常参与胞外结构域的脱落,要么释放例如生长因子,要么通过调节性膜内蛋白水解引发进一步的细胞内信号传导。主要基于在载体细胞中的过表达研究,其中三种,即ADAMs 9、10和17,被认为可作为淀粉样前体蛋白(APP)的α-分泌酶。引人注目的是,从那时起,这个作用在某种程度上仍然定义不明确,因为在ADAM9缺陷神经元中APP的加工未改变,而且ADAM10缺陷的小鼠胚胎成纤维细胞中α-分泌酶活性充其量也只是表现出高度可变的降低。然而,在过去几年中,许多其他底物已与这三种脱落酶相关联,在某些情况下,它们的切割对生物体似乎比对APP的加工更为重要。最值得注意的是,ADAM17基因敲除小鼠的围产期致死表型主要是由于生长因子脱落的丧失,而ADAM10缺陷的更早的致命影响表现出Notch信号传导功能障碍的关键特征,并且可能还有钙黏蛋白加工缺陷。在这篇综述中,我们将总结已发表的关于所有三种ADAMs的“非APP”功能的数据,当试图通过增加它们的表达和/或活性来治疗阿尔茨海默病时,对这些数据的进一步评估可能至关重要。由于ADAM10和ADAM17的基因敲除仅对它们在(早期)发育中的作用有参考价值,而一些最近确定的新底物在正常和/或患病的成年生物体中也起着关键作用,因此对条件性基因敲除模型的研究对于全面表征(候选)α-分泌酶的完整功能组合及其临床相关性至关重要,其临床相关性可能远远超出阿尔茨海默病。