Universität zu Köln

Dafinger, Claudia, Mandel, Amrei M., Braun, Alina, Goebel, Heike, Burgmaier, Kathrin, Massella, Laura, Mastrangelo, Antonio, Doetsch, Joerg, Benzing, Thomas, Weimbs, Thomas, Schermer, Bernhard and Liebau, Max C. (2020). The carboxy-terminus of the human ARPKD protein fibrocystin can control STAT3 signalling by regulating SRC-activation. J. Cell. Mol. Med., 24 (24). S. 14633 - 14639. HOBOKEN: WILEY. ISSN 1582-4934

Full text not available from this repository.

Abstract

Autosomal recessive polycystic kidney disease (ARPKD) is mainly caused by variants in the PKHD1 gene, encoding fibrocystin (FC), a large transmembrane protein of incompletely understood cellular function. Here, we show that a C-terminal fragment of human FC can suppress a signalling module of the kinase SRC and signal transducer and activator of transcription 3 (STAT3). Consistently, we identified truncating genetic variants specifically affecting the cytoplasmic tail in ARPKD patients, found SRC and the cytoplasmic tail of fibrocystin in a joint dynamic protein complex and observed increased activation of both SRC and STAT3 in cyst-lining renal epithelial cells of ARPKD patients.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Dafinger, Claudia UNSPECIFIED UNSPECIFIED UNSPECIFIED Mandel, Amrei M. UNSPECIFIED UNSPECIFIED UNSPECIFIED Braun, Alina UNSPECIFIED UNSPECIFIED UNSPECIFIED Goebel, Heike UNSPECIFIED UNSPECIFIED UNSPECIFIED Burgmaier, Kathrin UNSPECIFIED UNSPECIFIED UNSPECIFIED Massella, Laura UNSPECIFIED UNSPECIFIED UNSPECIFIED Mastrangelo, Antonio UNSPECIFIED UNSPECIFIED UNSPECIFIED Doetsch, Joerg UNSPECIFIED UNSPECIFIED UNSPECIFIED Benzing, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Weimbs, Thomas UNSPECIFIED UNSPECIFIED UNSPECIFIED Schermer, Bernhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Liebau, Max C. UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-314189
DOI:	10.1111/jcmm.16014
Journal or Publication Title:	J. Cell. Mol. Med.
Volume:	24
Number:	24
Page Range:	S. 14633 - 14639
Date:	2020
Publisher:	WILEY
Place of Publication:	HOBOKEN
ISSN:	1582-4934
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language POLYCYSTIC KIDNEY-DISEASE; AUTOSOMAL-DOMINANT; ADHESION Multiple languages Cell Biology; Medicine, Research & Experimental Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/31418