2026-05-22T00:27:23Zhttps://repisalud.isciii.es/rest/oai/request

oai:repisalud.isciii.es:20.500.12105/131272025-04-03T18:26:16Zcom_20.500.12105_2052com_20.500.12105_2051col_20.500.12105_19616

00925njm 22002777a 4500 dc Tumpara, Srinu author Ballmaier, Matthias author Wrenger, Sabine author König, Mandy author Lehmann, Matthias author Lichtinghagen, Ralf author Martinez-Delgado, Beatriz author Korenbaum, Elena author DeLuca, David S author Jedicke, Nils author Welte, Tobias author Fromme, Malin author Strnad, Pavel author Stolk, Jan author Janciauskiene, Sabina author 2021-05-18 The CX3CR1 (chemokine (C-X3-C motif) receptor 1) expression levels on immune cells have significant importance in maintaining tissue homeostasis under physiological and pathological conditions. The factors implicated in the regulation of CX3CR1 and its specific ligand CX3CL1 (fractalkine) expression remain largely unknown. Recent studies provide evidence that host`s misfolded proteins occurring in the forms of polymers or amyloid fibrils can regulate CX3CR1 expression. Herein, a novel example demonstrates that polymers of human ZZ alpha-1 antitrypsin (Z-AAT) protein, resulting from its conformational misfolding due to the Z (Glu342Lys) mutation in SERPINA1 gene, strongly lower CX3CR1 mRNA expression in human PBMCs. This parallels with increase of intracellular levels of CX3CR1 and Z-AAT proteins. Presented data indicate the involvement of the CX3CR1 pathway in the Z-AAT-related disorders and further support the role of misfolded proteins in CX3CR1 regulation. Elife. 2021 May 18;10:e64881. 10.7554/eLife.64881 2050-084X ELife 34002692 http://hdl.handle.net/20.500.12105/13127 Cell biology Human Medicine Polymerization of misfolded Z alpha-1antitrypin protein lowers CX3CR1 expression in human PBMCs