Generation and characterization of LANP/pp32 null mice
Generation and characterization of LANP/pp32 null mice
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DOI：
10.1128/mcb.24.8.3140-3149.2004
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发表时间：
2004-04-01
影响因子：
5.3
通讯作者：
Zoghbi, HY
中科院分区：
文献类型：
--
作者：
Opal, P;Garcia, JJ;Zoghbi, HY
富含亮氨酸的酸性核蛋白（LANP）属于进化上保守的蛋白质家族，其特征在于氨基末端富含亮氨酸残基，随后是羧基末端酸性尾。LANP参与调节多种细胞过程，包括RNA转运、转录、凋亡、囊泡运输和细胞内信号传导。这种蛋白质在发育中的小脑中大量表达，也被假设在小脑形态发生中发挥作用。LANP也与疾病生物学有关，既作为脊髓小脑共济失调I型的毒性介质，又作为乳腺癌和前列腺癌的肿瘤抑制因子。为了更好地了解这种多方面蛋白质的功能，我们产生了缺乏LANP的小鼠。令人惊讶的是，这些小鼠是可行的和可繁殖的。此外，我们无法辨别任何主要器官系统的任何紊乱，包括我们详细研究过的神经系统。总的来说，我们的研究结果指出，LANP的功能冗余的功能，最有可能提供其密切相关的家庭成员。
The leucine-rich acidic nuclear protein (LANP) belongs to a family of evolutionarily conserved proteins that are characterized by an amino-terminal domain rich in leucine residues followed by a carboxy-terminal acidic tail. LANP has been implicated in the regulation of a variety of cellular processes including RNA transport, transcription, apoptosis, vesicular trafficking, and intracellular signaling. Abundantly expressed in the developing cerebellum, this protein has also been hypothesized to play a role in cerebellar morphogenesis. LANP has been implicated in disease biology as well, both as a mediator of toxicity in spinocerebellar ataxia type I and as a tumor suppressor in cancers of the breast and prostate. To better understand the function of this multifaceted protein, we have generated mice lacking LANP. Surprisingly, these mice are viable and fertile. In addition we could not discern any derangements in any of the major organ systems, including the nervous system, which we have studied in detail. Overall our results point to a functional redundancy of LANP's function, most likely provided by its closely related family members.