2024-03-28T19:58:12Zhttp://repisalud.isciii.es/oai/requestoai:repisalud.isciii.es:20.500.12105/131272023-10-31T12:24:49Zcom_20.500.12105_2102com_20.500.12105_2052com_20.500.12105_2051col_20.500.12105_2103
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
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.
http://hdl.handle.net/20.500.12105/13127
34002692
10.7554/eLife.64881
2050-084X
ELife
Cell biology
Human
Medicine
Polymerization of misfolded Z alpha-1antitrypin protein lowers CX3CR1 expression in human PBMCs