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帕西拉(Pasilla)是人类Nova-1和Nova-2蛋白在果蝇中的同源物,是唾液腺正常分泌所必需的。

pasilla, the Drosophila homologue of the human Nova-1 and Nova-2 proteins, is required for normal secretion in the salivary gland.

作者信息

Seshaiah P, Miller B, Myat M M, Andrew D J

机构信息

Department of Cell Biology and Anatomy, The Johns Hopkins University School of Medicine, 725 N. Wolfe Street, Baltimore, Maryland 21205-2196, USA.

出版信息

Dev Biol. 2001 Nov 15;239(2):309-22. doi: 10.1006/dbio.2001.0429.

Abstract

From a screen for genes expressed and required in the Drosophila salivary gland, we identified pasilla (ps), which encodes a set of proteins most similar to human Nova-1 and Nova-2. Nova-1 and Nova-2 are nuclear RNA-binding proteins normally expressed in the CNS where they directly regulate splicing. In patients suffering from paraneoplastic opsoclonus myoclonus ataxia (POMA), Nova-1 and Nova-2 proteins are present as auto-antigens. Consistent with a role in splicing, PS is localized to nuclear puncta. The salivary glands of ps mutants internalize normally and maintain epithelial polarity. However, the mutant salivary glands develop irregularities in overall morphology and have defects in apical secretion. The secretory defects in ps mutants provide a potential mechanism for the loss of motor function observed in POMA patients.

摘要

通过对果蝇唾液腺中表达和必需的基因进行筛选,我们鉴定出了pasilla(ps),它编码一组与人类Nova-1和Nova-2最相似的蛋白质。Nova-1和Nova-2是通常在中枢神经系统中表达的核RNA结合蛋白,它们在中枢神经系统中直接调节剪接。在患有副肿瘤性眼阵挛性肌阵挛性共济失调(POMA)的患者中,Nova-1和Nova-2蛋白作为自身抗原存在。与在剪接中的作用一致,PS定位于核斑点。ps突变体的唾液腺正常内化并维持上皮极性。然而,突变体唾液腺在整体形态上出现异常,并且在顶端分泌方面存在缺陷。ps突变体中的分泌缺陷为POMA患者中观察到的运动功能丧失提供了一种潜在机制。

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