Bastianelli Enrico
ProSkelia Pharmaceuticals, Romainville, France.
Cerebellum. 2003;2(4):242-62. doi: 10.1080/14734220310022289.
Calcium plays a fundamental role in the cell as second messenger and is principally regulated by calcium-binding proteins. Although these proteins share in common their ability to bind calcium, they belong to different subfamilies. They present, in general, specific developmental and distribution patterns. Most Purkinje cells express the fast and slow calcium buffer proteins calbindin-D28k and parvalbumin, whereas basket, stellate and Golgi cells the slow buffer parvalbumin only. They are, almost all, calretinin negative. Granule, Lugaro and unipolar brush cells present an opposite immunoreactivity profile, most of them being calretinin positive while lacking calbindin-D28k and parvalbumin. The developmental pattern of appearance of these proteins seems to follow the maturation of neurons. Calbindin-D28k appears early, shortly after cessation of mitosis when neurons become ready to start migration and differentiation while parvalbumin is expressed later in parallel with an increase in neuronal activity. The other proteins are generally detected later. During development, some of these proteins, like calretinin, are transiently expressed in specific cellular subpopulations. The function of these proteins is not fully understood, although strong evidence supports a prominent role in physiological settings with altered calcium concentrations. These proteins regulate and are regulated by intracellular calcium level. For example, they may directly or indirectly enable sensitization or desensitization of calcium channels, and may further block calcium entry into the cells, like the calcium-sensor proteins, that have been shown to be potent and specific modulators of ion channels, which may allow for feedback control of current function and hence signaling. The absence of calcium buffer proteins results in marked abnormalities in cell firing; with alterations in simple and complex spikes or transformation of depressing synapses into facilitating synapses. Calcium-binding protein implication in resistance to degeneration is still a controversial issue. Neurons rich in calcium-binding proteins, especially calbindin-D28k and parvalbumin, seem to be relatively resistant to degeneration in a variety of acute and chronic disorders. However other data support that an absence of calcium-binding proteins may also have a neuroprotective effect. It is not unlikely that neurons may face a dual action mechanism where a decrease in calcium-binding proteins has a first short-term beneficial effect while it becomes detrimental for the cell over the long term.
钙作为第二信使在细胞中发挥着重要作用,主要由钙结合蛋白调节。尽管这些蛋白质都具有结合钙的能力,但它们属于不同的亚家族。一般来说,它们呈现出特定的发育和分布模式。大多数浦肯野细胞表达快速和慢速钙缓冲蛋白钙结合蛋白-D28k和小白蛋白,而篮状细胞、星状细胞和高尔基细胞仅表达慢速缓冲蛋白小白蛋白。几乎所有这些细胞都对钙视网膜蛋白呈阴性。颗粒细胞、卢加罗细胞和单极刷状细胞呈现相反的免疫反应谱,它们中的大多数对钙视网膜蛋白呈阳性,而缺乏钙结合蛋白-D28k和小白蛋白。这些蛋白质出现的发育模式似乎遵循神经元的成熟过程。钙结合蛋白-D28k出现较早,在有丝分裂停止后不久,当神经元准备开始迁移和分化时出现,而小白蛋白则在神经元活动增加的同时稍后表达。其他蛋白质通常在更晚的时候被检测到。在发育过程中,这些蛋白质中的一些,如钙视网膜蛋白,会在特定的细胞亚群中短暂表达。尽管有强有力的证据支持这些蛋白质在钙浓度改变的生理环境中发挥重要作用,但它们的功能尚未完全了解。这些蛋白质调节细胞内钙水平并受其调节。例如,它们可能直接或间接使钙通道敏感或脱敏,并可能进一步阻止钙进入细胞,就像钙传感器蛋白一样,已被证明是离子通道的有效和特异性调节剂,这可能允许对电流功能进行反馈控制,从而实现信号传导。钙缓冲蛋白的缺失会导致细胞放电出现明显异常,简单和复杂的锋电位发生改变,或者抑制性突触转变为易化性突触。钙结合蛋白与抗变性的关系仍然是一个有争议的问题。富含钙结合蛋白,尤其是钙结合蛋白-D28k和小白蛋白的神经元,在各种急性和慢性疾病中似乎相对抗变性。然而,其他数据支持缺乏钙结合蛋白也可能具有神经保护作用。神经元可能面临一种双重作用机制,即钙结合蛋白的减少在短期内有有益作用,但从长期来看对细胞是有害的,这并非不可能。