Universität zu Köln

Staubach, Simon, Mueller, Stefan, Pekmez, Murat and Hanisch, Franz-Georg (2017). Classical Galactosemia: Insight into Molecular Pathomechanisms by Differential Membrane Proteomics of Fibroblasts under Galactose Stress. J. Proteome Res., 16 (2). S. 516 - 528. WASHINGTON: AMER CHEMICAL SOC. ISSN 1535-3907

Full text not available from this repository.

Abstract

Classical galactosemia, a hereditary metabolic disease caused by the deficiency of galactose-1-phosphate uridyltransferase (GALT; EC 2.7.712), results in an impaired galactose metabolism and serious long-term developmental affection of the CNS and ovaries, potentially related in part to endogenous galactose-induced protein dysglycosylation. In search for galactose-induced changes in membrane raft proteomes of GALT-deficient cells, we performed differential analyses of lipid rafts from patient-derived (Q) and sex- and age-matched control fibroblasts (H) in the presence or absence of the stressor. Label based proteomics revealed of the total 454 (female) or 678 (male) proteins a proportion of similar to 12% in at least one of four relevant ratios as fold-changed. GALT(-) cell-specific effects in the absence of stressor revealed cell-model-dependent affection of biological processes related to protein targeting to the plasma membrane (female) or to cellular migration (male). However, a series of common galactose-induced effects were observed, among them the strongly increased ER-stress marker GRP78 and calreticulin involved in N-glycoprotein quality control. The membrane-anchored N-glycoprotein receptor CD109 was concertedly decreased under galactose-stress together with cadherin-13, GLIPR1, glypican-1, and semaphorin-7A. A series of proteins showed opposite fold-changes in the two cell models, whereas others fluctuated in only one of the two models.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Staubach, Simon UNSPECIFIED UNSPECIFIED UNSPECIFIED Mueller, Stefan UNSPECIFIED UNSPECIFIED UNSPECIFIED Pekmez, Murat UNSPECIFIED UNSPECIFIED UNSPECIFIED Hanisch, Franz-Georg UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-241174
DOI:	10.1021/acs.jproteome.6b00658
Journal or Publication Title:	J. Proteome Res.
Volume:	16
Number:	2
Page Range:	S. 516 - 528
Date:	2017
Publisher:	AMER CHEMICAL SOC
Place of Publication:	WASHINGTON
ISSN:	1535-3907
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language URINARY EXOVESICLES; T-CADHERIN; H-CADHERIN; COMPLEX; CELLS; EXPRESSION; GLYCOSYLATION; GLYCOPROTEINS; GRP78 Multiple languages Biochemical Research Methods Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/24117