Role of IP3 receptor in CA2+ signaling and development and differentiation

肌醇1，4，5-三磷酸（IP 3）受体在多种细胞类型中充当IP 3门控的Ca 2+释放通道。YP 1 IP 3受体（IP 3R）是中枢神经系统中IP 3R家族的主要神经元成员，主要富集在小脑浦肯野细胞中，但也集中在海马CA 1区、尾壳核和大脑皮质中的神经元中。在此我们报道了大多数通过基因打靶产生的IP 3R 1缺陷小鼠在子宫内死亡，并且出生的动物具有严重的共济失调和强直性或强直-阵挛性癫痫发作，并且死于衰老。脑电图显示他们患有癫痫，表明IP 3R 1对正常的大脑功能至关重要。然而，通过光学显微镜观察IP 3R 1缺陷小鼠的脑和外周组织的苏木精-伊红染色未显示异常，并且IP 3R 1缺陷小鼠的小脑浦肯野细胞的独特电生理特性未严重受损。

The inositol 1,4,5-trisphosphate (IP3) receptor acts as an IP3-gated Ca2+ release channel in a variety of cell types. Yupe 1 IP3 receptor (IP3R) is the major neuronal member of the IP3R family in the central nervous system, predominantly enriched in cerebellar Purkinje cells but also concentrated in neurons in the hippocampal CA1 region, caudate-putamen, and cerebral cortex. Here we report that most IP3R1- deficient mice geberated by gene targeting die in utero, and born animals have severe ataxia and tonic or tonic-clonic seizures and die by the wearing geriod. An electroncephalogram showed that they suffer from epilepsy, indicating that IP3R1 is essential for proper brain function. However, observation by light microscope of the haematoxylin-eosin staining of the brain and peripheral tissues of IP3R1-deficient mice showed no abnormality, and the unique electrophysiological properties of the cerebellar Purkinje cells of IP3R1-deficient mice were not severely impaired.