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与自发性冠状动脉夹层 (SCAD) 相关的肌腱蛋白-1 变体强调了多功能支架蛋白中的即使是细微的变化也可能在疾病中表现出来。

Talin-1 variants associated with spontaneous coronary artery dissection (SCAD) highlight how even subtle changes in multi-functional scaffold proteins can manifest in disease.

机构信息

Faculty of Medicine and Health Technology, Tampere University, Arvo Ylpön katu, 33520 Tampere, Finland.

Department of Biochemistry, Cell & Systems Biology, Institute of Systems, Molecular & Integrative Biology, University of Liverpool, Crown Street, Liverpool L69 7ZB, United States.

出版信息

Hum Mol Genet. 2024 Nov 5;33(21):1846-1857. doi: 10.1093/hmg/ddae120.

Abstract

Variants of talin-1 (TLN1) have recently been linked with spontaneous coronary artery dissection (SCAD) a condition where a tear can form in the wall of a heart artery necessitating immediate medical care. One talin-1 variant, A2013T, has an extensive familial pedigree of SCAD, which led to the screening for, and identification of, further talin-1 variants in SCAD patients. Here we evaluated these variants with commonly used pathogenicity prediction tools and found it challenging to reliably classify SCAD-associated variants, even A2013T where the evidence of a causal role is strong. Using biochemical and cell biological methods, we show that SCAD-associated variants in talin-1, which would typically be classified as non-pathogenic, still cause a measurable impact on protein structure and cell behaviour, including cell movement and wound healing capacity. Together, this indicates that even subtle variants in central mechanosensitive adapter proteins, can give rise to significant health impacts at the individual level, suggesting the need for a possible re-evaluation of the scoring criteria for pathogenicity prediction for talin variants.

摘要

塔林-1(TLN1)的变体最近与自发性冠状动脉夹层(SCAD)有关,在这种情况下,心脏动脉壁会出现撕裂,需要立即进行医疗护理。塔林-1 的一种变体 A2013T 在 SCAD 患者中有广泛的家族谱系,这导致了对 SCAD 患者中进一步的塔林-1 变体的筛选和鉴定。在这里,我们使用常用的致病性预测工具评估了这些变体,发现即使是 A2013T,其因果作用的证据很强,也很难可靠地区分与 SCAD 相关的变体。使用生化和细胞生物学方法,我们表明,塔林-1 中的 SCAD 相关变体通常被归类为非致病性,但仍会对蛋白质结构和细胞行为产生可测量的影响,包括细胞运动和伤口愈合能力。总之,这表明即使是中央机械敏感衔接蛋白中的细微变体,也可能在个体水平上产生重大的健康影响,这表明可能需要重新评估塔林变体致病性预测的评分标准。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9dc0/11540920/1d9d26b28693/ddae120f1.jpg

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