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关于神经元蜡样脂褐质沉积症相关基因(CLN1、CLN2、CLN3、CLN5)基因表达模式的特征数据及其与中间神经元和神经传递标志物(小白蛋白和生长抑素)的关联。

Data on characterizing the gene expression patterns of neuronal ceroid lipofuscinosis genes: CLN1, CLN2, CLN3, CLN5 and their association to interneuron and neurotransmission markers: Parvalbumin and Somatostatin.

作者信息

Minye Helena M, Fabritius Anna-Liisa, Vesa Jouni, Peltonen Leena

机构信息

Department of Human Genetics, David Geffen School of Medicine, University of California, Los Angeles, CA, USA.

Department of Applied Biology, University of Helsinki, Finland; Department of Human Genetics, David Geffen School of Medicine, University of California, Los Angeles, CA, USA.

出版信息

Data Brief. 2016 Jun 23;8:741-9. doi: 10.1016/j.dib.2016.06.027. eCollection 2016 Sep.

DOI:10.1016/j.dib.2016.06.027
PMID:27508227
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4950137/
Abstract

The article contains raw and analyzed data related to the research article "Neuronal ceroid lipofuscinosis genes, CLN2, CLN3, CLN5 are spatially and temporally co-expressed in a developing mouse brain" (Fabritius et al., 2014) [1]. The processed data gives an understanding of the development of the cell types that are mostly affected by defective function of CLN proteins, timing of expression of CLN1, CLN2, CLN3 and CLN5 genes in a murine model. The data shows relationship between the expression pattern of these genes during neural development. Immunohistochemistry was used to identify known interneuronal markers for neurotransmission and cell proliferation: parvalbumin, somatostatin subpopulations of interneurons. Non-radioactive in-situ hybridization detected CLN5 mRNA in the hippocampus. Throughout the development strong expression of CLN genes were identified in the germinal epithelium and in ventricle regions, cortex, hippocampus, and cerebellum. This provides supportive evidence that CLN1, CLN2, CLN3 and CLN5 genes may be involved in synaptic pruning.

摘要

本文包含与研究论文《神经元蜡样脂褐质沉积症基因CLN2、CLN3、CLN5在发育中的小鼠大脑中时空共表达》(法布里修斯等人,2014年)[1]相关的原始数据和分析数据。处理后的数据有助于了解受CLN蛋白功能缺陷影响最大的细胞类型的发育情况,以及CLN1、CLN2、CLN3和CLN5基因在小鼠模型中的表达时间。数据显示了这些基因在神经发育过程中的表达模式之间的关系。免疫组织化学用于鉴定已知的用于神经传递和细胞增殖的中间神经元标志物:小白蛋白、生长抑素中间神经元亚群。非放射性原位杂交检测到海马体中CLN5 mRNA。在整个发育过程中,在生发上皮、脑室区域、皮质、海马体和小脑中均鉴定出CLN基因的强表达。这提供了支持性证据,表明CLN1、CLN2、CLN3和CLN5基因可能参与突触修剪。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/40e2b3f84872/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/d57ec2dc7fcd/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/eaad1784fe5e/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/75d811cd27be/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/5c5cd7bef377/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/3936eac0afdc/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/40e2b3f84872/gr6.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/d57ec2dc7fcd/gr1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/eaad1784fe5e/gr2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/75d811cd27be/gr3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/5c5cd7bef377/gr4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/3936eac0afdc/gr5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4b44/4950137/40e2b3f84872/gr6.jpg

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2
A mouse model for Finnish variant late infantile neuronal ceroid lipofuscinosis, CLN5, reveals neuropathology associated with early aging.一种针对芬兰变异型晚发性婴儿神经元蜡样脂褐质沉积症(CLN5)的小鼠模型揭示了与早衰相关的神经病理学特征。
Hum Mol Genet. 2004 Dec 1;13(23):2893-906. doi: 10.1093/hmg/ddh312. Epub 2004 Sep 30.
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