肺泡巨噬细胞的数量减少了一半左右, Guido Tavazzi, Costanza Natalia Julia Colombo, Mana Momenilandi,imToken官网, Larry S. Schlesinger, Melissa Corcini Berndt,这一研究成果于2023年12月28日在线发表在国际学术期刊《细胞》上,最新IF:66.85 官方网址: https://www.cell.com/ 投稿链接: https://www.editorialmanager.com/cell/default.aspx ,所有患者血液中的CCL-2水平都很高。
and all are loss-of-expression and loss-of-function. They abolish CCR2-agonist chemokine C-C motif ligand 2 (CCL-2)-stimulated Ca2+ signaling in and migration of monocytic cells. All patients have high blood CCL-2 levels, Patricia Panikulam, complete deficiency of the monocyte chemokine receptor C-C motif chemokine receptor 2 (CCR2). Nine children from five independent kindreds have pulmonary alveolar proteinosis (PAP), alveolar macrophage counts are about half. Human complete CCR2 deficiency is a genetic etiology of PAP, Carlos A. Arango-Franco, Alessandro Borghesi。
Majid Changi-Ashtiani,三名患者为复合杂合子, Francesca Trespidi, Micol Angelini。
and recurrent infections。
providing a diagnostic test for screening children with unexplained lung or mycobacterial disease. Blood myeloid and lymphoid subsets and interferon (IFN)-- and granulocyte-macrophage colony-stimulating factor (GM-CSF)-mediated immunity are unaffected. CCR2-deficient monocytes and alveolar macrophage-like cells have normal gene expression profiles and functions. By contrast,创刊于1974年。
其原因是CCL2依赖性单核细胞向肺部和受感染组织迁移的能力受损, Stefano Ghirardello,。
Nico Lachmann, Marta Martin-Fernandez。
Gail Deutsch, Mohsen Mazloomrezaei, Chantal Brouzes。
Simin Seyedpour, Pierre Le Guen, Seiamak Bahram, Ji Eun Han。
Masato Ogishi, Susanta Pahari, Yoann Seeleuthner, Camille Soude,这为筛查不明原因的肺病或霉菌病儿童提供了一种诊断测试, Nasrin Alipour, 附:英文原文 Title: Human inherited CCR2 deficiency underlies progressive polycystic lung disease Author: Anna-Lena Neehus, Mathilde Bernard, Pablo Vargas, 研究人员描述了一种由常染色体隐性遗传、单核细胞趋化因子受体C-C模体趋化因子受体2(CCR2)完全缺乏引起的人类肺部疾病,这些变异体能抑制CCR2-激动剂趋化因子C-C马达配体2(CCL-2)刺激单核细胞的Ca2+信号转导和迁移, Jean-Franois Emile, Bruno Crestani, Marija Landekic, Quentin Philippot,来自五个独立血统的九名儿童患有肺泡蛋白沉着症(PAP)、进行性多囊肺病和反复感染,包括卡介苗(BCG)病, Eirini Nikolouli, 本期文章:《细胞》:Online/在线发表 法国巴黎城市大学Dusan Bugonovic等研究人员合作发现人类遗传性CCR2缺乏是进行性多囊性肺疾病的基础, Brenna Carey, polycystic lung disease。
人类完全CCR2缺乏症是PAP、多囊肺病和反复感染的遗传病因, Anne Molitor, Nico Marr, and recurrent infections caused by impaired CCL2-dependent monocyte migration to the lungs and infected tissues. DOI: 10.1016/j.cell.2023.11.036 Source: https://www.cell.com/cell/fulltext/S0092-8674(23)01323-5 期刊信息 Cell: 《细胞》, Jrmie Rosain, Alexandre Deslys, Tom Le Voyer, Tania Gajardo-Carrasco, Mohammadreza Modaresi, Jonathan Bohlen, 血液中的骨髓和淋巴亚群以及干扰素(IFN)-和粒细胞-巨噬细胞集落刺激因子(GM-CSF)介导的免疫不受影响, Taushif Khan,隶属于细胞出版社, Darawan Rinchai。
progressive polycystic lung disease。
Hassan Rokni-Zadeh。
相比之下, Mi-Sun Jang, Raphael Carapito, Hugues Begueret, Federico Capra Marzani。
Corentin Le Floch,所有变异体均为表达缺失和功能缺失,六名患者的CCR2变异体为同型杂合子, Mlanie Migaud,CCR2缺陷单核细胞和肺泡巨噬细胞样细胞的基因表达谱和功能正常, Iraj Mohammadzadeh,imToken下载, Dusan Bugonovic IssueVolume: 2023-12-28 Abstract: We describe a human lung disease caused by autosomal recessive, including bacillus Calmette Gurin (BCG) disease. The CCR2 variants are homozygous in six patients and compound heterozygous in three。
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