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罕见黏多糖贮积症中的基因聚类分离。

Separating gene clustering in the rare mucopolysaccharidosis disease.

机构信息

Faculty of Computer Science, Białystok University of Technology, Białystok, Poland.

Institute of Biocybernetics and Biomedical Engineering, Polish Academy of Sciences, Warsaw, Poland.

出版信息

J Appl Genet. 2022 May;63(2):361-368. doi: 10.1007/s13353-022-00691-2. Epub 2022 Mar 24.

DOI:10.1007/s13353-022-00691-2
PMID:35322332
Abstract

Rare disease datasets are typically structured such that a small number of patients (cases) are represented by multidimensional feature vectors. In this report, we considered a rare disease, mucopolysaccharidosis (MPS). This disease is divided into 11 types and subtypes, depending on the genetic defect, type of deficient enzyme, and nature of accumulated glycosaminoglycan(s). Among them, 7 types are known as possibly neuronopathic and 4 are non-neuronopathic, and in the case of the former group, prediction of the course of the disease is crucial for patient's treatment and the management. Here, we have used transcriptomic data available for one patient from each MPS type/subtype. The approach to gene grouping considered by us was based on the minimization of the perceptron criterion in the form of convex and piecewise linear function (CPL). This approach allows designing complexes of linear classifiers on the basis of small samples of multivariate vectors. As a result, distinguishing neuronopathic and non-neuronopathic forms of MPS was possible on the basis of bioinformatic analysis of gene expression patterns where each MPS type was represented by only one patient. This approach can be potentially used also for assessing other features of patients suffering from rare diseases, for which large body of data (like transcriptomic data) is available from only one or a few representatives.

摘要

罕见病数据集通常采用多维特征向量的方式表示少量患者(病例)。在本报告中,我们考虑了一种罕见病,黏多糖贮积症(MPS)。这种疾病根据遗传缺陷、缺乏的酶类型以及积累的糖胺聚糖的性质分为 11 种类型和亚型。其中,7 种为可能神经病变型,4 种为非神经病变型,对于前者,预测疾病进程对于患者的治疗和管理至关重要。在这里,我们使用了每种 MPS 类型/亚型的一位患者的转录组数据。我们考虑的基因分组方法基于感知器准则的最小化,形式为凸和分段线性函数(CPL)。这种方法允许在小样本的多元向量的基础上设计线性分类器的组合。结果,在对基因表达模式进行生物信息学分析的基础上,我们能够区分 MPS 的神经病变型和非神经病变型,其中每种 MPS 类型仅由一位患者代表。这种方法也可以潜在地用于评估其他患有罕见病的患者的特征,对于这些患者,只有一个或几个代表有大量的数据(如转录组数据)可用。

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引用本文的文献

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本文引用的文献

1
A cDNA analysis disclosed the discordance of genotype-phenotype correlation in a patient with attenuated MPS II and a 76-base deletion in the gene for iduronate-2-sulfatase.一项cDNA分析揭示了一名黏多糖贮积症II型症状减轻患者的基因型-表型相关性不一致,该患者的艾杜糖-2-硫酸酯酶基因存在76个碱基的缺失。
Mol Genet Metab Rep. 2020 Dec 10;25:100692. doi: 10.1016/j.ymgmr.2020.100692. eCollection 2020 Dec.
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The mutational spectrum of hunter syndrome reveals correlation between biochemical and clinical profiles in Tunisian patients.亨特综合征的突变谱揭示了突尼斯患者生化特征与临床特征之间的相关性。
BMC Med Genet. 2020 May 24;21(1):111. doi: 10.1186/s12881-020-01051-9.
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Genotype-phenotype relationships in mucopolysaccharidosis type I (MPS I): Insights from the International MPS I Registry.
黏多糖贮积症 I 型(MPS I)的基因型-表型关系:国际 MPS I 注册中心的见解。
Clin Genet. 2019 Oct;96(4):281-289. doi: 10.1111/cge.13583. Epub 2019 Jul 2.
4
Mutation spectrum and pivotal features for differential diagnosis of Mucopolysaccharidosis IVA patients with severe and attenuated phenotype.MucopolysaccharidosisIVA 患者严重和轻度表型的突变谱及鉴别诊断的关键特征。
Gene. 2019 Jul 1;704:59-67. doi: 10.1016/j.gene.2019.04.026. Epub 2019 Apr 11.
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Correlation between severity of mucopolysaccharidoses and combination of the residual enzyme activity and efficiency of glycosaminoglycan synthesis.黏多糖贮积症严重程度与残余酶活性及糖胺聚糖合成效率之间的相关性。
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