螺内酯抑制NLRP1炎性小体并减轻钻石黑范贫血中存在缺陷的红细胞生成。 - Suppr

Spironolactone inhibits the NLRP1 inflammasome and alleviates defective erythropoiesis in Diamond-Blackfan anemia.

作者信息

Rodríguez-Ruiz Lola, Lozano-Gil Juan M, Ocaña-Esparza María, Conesa-Hernández Ana M, Pérez-Oliva Ana B, Fuster José L, Jérez Andrés, Murillo-Sanjuán Laura, Díaz-de-Heredia Cristina, López-de-Hontanar Guzmán, Sevilla Julián, García-Moreno Diana, Cayuela María L, Martínez-López Alicia, Tyrkalska Sylwia D, Mulero Victoriano

机构信息

Departmento de Biología Celular e Histología Facultad de Biología, Universidad de Murcia Murcia Spain.

Instituto Murciano de Investigación Biosanitaria (IMIB)-Pascual Parrilla Murcia Spain.

出版信息

Hemasphere. 2025 Apr 17;9(4):e70131. doi: 10.1002/hem3.70131. eCollection 2025 Apr.

DOI:10.1002/hem3.70131

PMID:40248129

原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12005213/

Abstract

摘要

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7e8c/12005213/c650a1393fde/HEM3-9-e70131-g001.jpg

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Spironolactone inhibits the NLRP1 inflammasome and alleviates defective erythropoiesis in Diamond-Blackfan anemia.螺内酯抑制NLRP1炎性小体并减轻钻石黑范贫血中存在缺陷的红细胞生成。

Hemasphere. 2025 Apr 17;9(4):e70131. doi: 10.1002/hem3.70131. eCollection 2025 Apr.

Enhanced alternative splicing of the FLVCR1 gene in Diamond Blackfan anemia disrupts FLVCR1 expression and function that are critical for erythropoiesis.钻石黑fan贫血中FLVCR1基因增强的可变剪接破坏了对红细胞生成至关重要的FLVCR1表达和功能。

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Diamond-Blackfan anemia RPL35A: a case report.钻石黑范贫血RPL35A：一例报告

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Diamond-Blackfan anemia: erythropoiesis lost in translation.先天性纯红细胞再生障碍性贫血：翻译过程中丢失的红细胞生成

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Deficiency of ribosomal protein S26, which is mutated in a subset of patients with Diamond Blackfan anemia, impairs erythroid differentiation.核糖体蛋白S26缺乏会损害红系分化，该蛋白在一部分先天性纯红细胞再生障碍性贫血患者中发生了突变。

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Hepcidin levels in Diamond-Blackfan anemia reflect erythropoietic activity and transfusion dependency.钻石黑范贫血患者的铁调素水平反映了红细胞生成活性和输血依赖性。

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The active component of ginseng, ginsenoside Rb1, improves erythropoiesis in models of Diamond-Blackfan anemia by targeting Nemo-like kinase.人参的活性成分人参皂苷 Rb1 通过靶向 Nemo 样激酶改善 Diamond-Blackfan 贫血模型中的红细胞生成。

J Biol Chem. 2021 Sep;297(3):100988. doi: 10.1016/j.jbc.2021.100988. Epub 2021 Jul 21.

Diamond blackfan anemia stem cells fail to repopulate erythropoiesis in NOD/SCID mice.先天性纯红细胞再生障碍性贫血干细胞无法在NOD/SCID小鼠中重新填充红细胞生成。

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Targeting of Calbindin 1 rescues erythropoiesis in a human model of Diamond Blackfan anemia.钙结合蛋白 1 靶向治疗可挽救 Diamond Blackfan 贫血症的人类模型中的红细胞生成。

Blood Cells Mol Dis. 2023 Sep;102:102759. doi: 10.1016/j.bcmd.2023.102759. Epub 2023 May 26.

本文引用的文献

ZAKα/P38 kinase signaling pathway regulates hematopoiesis by activating the NLRP1 inflammasome.ZAKα/P38激酶信号通路通过激活NLRP1炎性小体来调节造血作用。

EMBO Mol Med. 2023 Oct 11;15(10):e18142. doi: 10.15252/emmm.202318142. Epub 2023 Sep 7.

Mechanism of filament formation in UPA-promoted CARD8 and NLRP1 inflammasomes.UPA 促进的 CARD8 和 NLRP1 炎性小体中丝状体形成的机制。

Nat Commun. 2021 Jan 8;12(1):189. doi: 10.1038/s41467-020-20320-y.

Diamond-Blackfan anemia. Diamond-Blackfan 贫血。

Blood. 2020 Sep 10;136(11):1262-1273. doi: 10.1182/blood.2019000947.

Inflammasome Regulates Hematopoiesis through Cleavage of the Master Erythroid Transcription Factor GATA1.炎症小体通过切割主红细胞转录因子 GATA1 调节造血。

Immunity. 2019 Jul 16;51(1):50-63.e5. doi: 10.1016/j.immuni.2019.05.005. Epub 2019 Jun 4.

Ribosome Levels Selectively Regulate Translation and Lineage Commitment in Human Hematopoiesis.核糖体水平在人类造血中选择性地调节翻译和谱系分化。

Cell. 2018 Mar 22;173(1):90-103.e19. doi: 10.1016/j.cell.2018.02.036. Epub 2018 Mar 15.

Spironolactone.螺内酯

J Pain Symptom Manage. 2017 Feb;53(2):288-292. doi: 10.1016/j.jpainsymman.2016.12.320. Epub 2016 Dec 23.

Differential actions of eplerenone and spironolactone on the protective effect of testosterone against cardiomyocyte apoptosis in vitro.依普利酮和螺内酯对睾酮体外抗心肌细胞凋亡保护作用的差异作用。

Rev Esp Cardiol. 2010 Jul;63(7):779-87. doi: 10.1016/s1885-5857(10)70162-6.

ESC Guidelines for the diagnosis and treatment of acute and chronic heart failure 2008: the Task Force for the Diagnosis and Treatment of Acute and Chronic Heart Failure 2008 of the European Society of Cardiology. Developed in collaboration with the Heart Failure Association of the ESC (HFA) and endorsed by the European Society of Intensive Care Medicine (ESICM).《2008年欧洲心脏病学会急性和慢性心力衰竭诊断与治疗指南》：欧洲心脏病学会2008年急性和慢性心力衰竭诊断与治疗特别工作组制定。与欧洲心脏病学会心力衰竭协会（HFA）合作编写，并得到欧洲重症医学学会（ESICM）认可。

Eur Heart J. 2008 Oct;29(19):2388-442. doi: 10.1093/eurheartj/ehn309. Epub 2008 Sep 17.

A comparison of the aldosterone-blocking agents eplerenone and spironolactone.醛固酮阻断剂依普利酮和螺内酯的比较。

Clin Cardiol. 2008 Apr;31(4):153-8. doi: 10.1002/clc.20324.

The evolution of aldosterone antagonists.醛固酮拮抗剂的演变

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Spironolactone inhibits the NLRP1 inflammasome and alleviates defective erythropoiesis in Diamond-Blackfan anemia.

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