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《葡萄糖-6-磷酸脱氢酶变异体在墨西哥人群中的功能和结构特征概述》。

An Overall View of the Functional and Structural Characterization of Glucose-6-Phosphate Dehydrogenase Variants in the Mexican Population.

机构信息

Laboratorio de Inmunoquímica, Hospital Infantil de México Federico Gómez, Secretaría de Salud, Mexico City 06720, Mexico.

Laboratorio de Nutrición Experimental, Instituto Nacional de Pediatría, Secretaría de Salud, Mexico City 04530, Mexico.

出版信息

Int J Mol Sci. 2023 Aug 11;24(16):12691. doi: 10.3390/ijms241612691.

Abstract

Glucose-6-phosphate dehydrogenase (G6PD) deficiency, affecting an estimated 500 million people worldwide, is a genetic disorder that causes human enzymopathies. Biochemical and genetic studies have identified several variants that produce different ranges of phenotypes; thus, depending on its severity, this enzymopathy is classified from the mildest (Class IV) to the most severe (Class I). Therefore, understanding the correlation between the mutation sites of G6PD and the resulting phenotype greatly enhances the current knowledge of enzymopathies' phenotypic and genotypic heterogeneity, which will assist both clinical diagnoses and personalized treatments for patients with G6PD deficiency. In this review, we analyzed and compared the structural and functional data from 21 characterized G6PD variants found in the Mexican population that we previously characterized. In order to contribute to the knowledge regarding the function and structure of the variants associated with G6PD deficiency, this review aimed to determine the molecular basis of G6PD and identify how these mutations could impact the structure, stability, and function of the enzyme and its relation with the clinical manifestations of this disease.

摘要

葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症影响着全球约 5 亿人,是一种导致人类酶病的遗传疾病。生化和遗传学研究已经确定了几种产生不同表型范围的变体;因此,根据其严重程度,这种酶病从最轻微(IV 类)到最严重(I 类)进行分类。因此,了解 G6PD 突变位点与表型之间的相关性,极大地增强了对酶病表型和基因型异质性的现有认识,这将有助于 G6PD 缺乏症患者的临床诊断和个体化治疗。在这篇综述中,我们分析和比较了我们之前在墨西哥人群中鉴定出的 21 种特征性 G6PD 变体的结构和功能数据。为了有助于了解与 G6PD 缺乏相关的变体的功能和结构,本综述旨在确定 G6PD 的分子基础,并确定这些突变如何影响酶的结构、稳定性和功能及其与该疾病临床表现的关系。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ccfe/10454679/2e03c4875595/ijms-24-12691-g003.jpg

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