Universität zu Köln

Esho, Temitope, Tufa, Sara F., Kobbe, Birgit, Wohl, Alexander P., Sengle, Gerhard, Paulsson, Mats ORCID: 0000-0002-6846-857X, Keene, Douglas R. and Wagener, Raimund (2022). Anchoring Cords: A Distinct Suprastructure in the Developing Skin. J. Invest. Dermatol., 142 (11). S. 2940 - 2951. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1523-1747

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Abstract

AMACO (VWA2 protein) is a basement membrane-associated protein secreted by epithelial cells. It is strongly expressed when invagination or budding occurs during development. AMACO associates with the Fraser complex, which when mutated causes Fraser syndrome, characterized by subepidermal blistering, cryptoph-thalmos, and syndactyly. The core Fraser complex proteins FRAS1, FREM1, and FREM2 localize at the dermal-epidermal junction and mediate adhesion to the underlying dermis during embryonic development. Earlier transmission electron microscopy studies of adult mouse skin showed clustered AMACO deposition below the lamina densa. In this study, we report a distinct cord-like suprastructure in the neonate dermis to which AMACO- and Fraser complex-associated proteins contribute. We propose anchoring cords to designate the suprastructure. Anchoring cords have a diameter of 60 nm when immunolabeled, originate from the basement membrane, and extend several microns into the dermis. In normal skin, they are evident after immunogold electron microscopy and are strikingly appreciated in thicker sections. In recessive dystrophic epidermolysis bullosa skin, they are directly visible where collagen VII anchoring fibrils are ablated. Immunofluorescence and coimmunoprecipitation of skin extracts identify a direct interaction of FREM2 and AMACO.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Esho, Temitope UNSPECIFIED UNSPECIFIED UNSPECIFIED Tufa, Sara F. UNSPECIFIED UNSPECIFIED UNSPECIFIED Kobbe, Birgit UNSPECIFIED UNSPECIFIED UNSPECIFIED Wohl, Alexander P. UNSPECIFIED UNSPECIFIED UNSPECIFIED Sengle, Gerhard UNSPECIFIED UNSPECIFIED UNSPECIFIED Paulsson, Mats UNSPECIFIED orcid.org/0000-0002-6846-857X UNSPECIFIED Keene, Douglas R. UNSPECIFIED UNSPECIFIED UNSPECIFIED Wagener, Raimund UNSPECIFIED UNSPECIFIED UNSPECIFIED
URN:	urn:nbn:de:hbz:38-668124
DOI:	10.1016/j.jid.2022.04.025
Journal or Publication Title:	J. Invest. Dermatol.
Volume:	142
Number:	11
Page Range:	S. 2940 - 2951
Date:	2022
Publisher:	ELSEVIER SCIENCE INC
Place of Publication:	NEW YORK
ISSN:	1523-1747
Language:	English
Faculty:	Unspecified
Divisions:	Unspecified
Subjects:	no entry
Uncontrolled Keywords:	Keywords Language FRASER-SYNDROME; BASEMENT-MEMBRANE; PROTEIN NEPHRONECTIN; VII COLLAGEN; IDENTIFICATION; DOMAIN; LOCALIZATION; COMPONENT; AMACO; CRYPTOPHTHALMOS Multiple languages Dermatology Multiple languages
URI:	http://kups.ub.uni-koeln.de/id/eprint/66812