Universität zu Köln

Pesch, Monika, Koenig, Sabrina and Aumailley, Monique ORCID: 0000-0002-4578-0966 (2017). Targeted Disruption of the Lama3 Gene in Adult Mice Is Sufficient to Induce Skin Inflammation and Fibrosis. J. Invest. Dermatol., 137 (2). S. 332 - 341. NEW YORK: ELSEVIER SCIENCE INC. ISSN 1523-1747

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Abstract

Genetic, clinical, and biochemical studies have shown that integrity of the dermal-epidermal junction requires a particular subset of laminins, that is, those containing the alpha 3 chain encoded by the Lama3 gene. Inherited mutations in the human gene or introduction of constitutive mutations in the mouse gene prevent expression of these laminins, causing junctional epidermolysis bullosa, a very severe, often lethal disorder characterized by detachment of the epidermis from the dermis. This has precluded in vivo functional analysis of alpha 3 chain-containing laminins, and it is still unknown whether and how they contribute to adult skin homeostasis. To address this question, we have disrupted the Lama3 gene in basal keratinocytes of adult mice. This led to the gradual disappearance of alpha 3 chain-containing laminins along the dermal-epidermal junction and formation of subepidermal blisters like in congenital junctional epidermis bullosa. The mice lose their nails and have bullae and erosions on the footpads. Because the blistering is restricted to the interfollicular epidermis, the animals do not lose the epidermis and are viable. There is abundant and scattered deposition of collagen VII on the dermal side of the blisters, inflammation, and development of skin fibrosis with extensive accumulation of interstitial and microfibrillar collagens.

Item Type:	Journal Article
Creators:	Creators Email ORCID ORCID Put Code Pesch, Monika UNSPECIFIED UNSPECIFIED UNSPECIFIED Koenig, Sabrina UNSPECIFIED UNSPECIFIED UNSPECIFIED Aumailley, Monique UNSPECIFIED orcid.org/0000-0002-4578-0966 UNSPECIFIED
URN:	urn:nbn:de:hbz:38-241440
DOI:	10.1016/j.jid.2016.07.040
Journal or Publication Title:	J. Invest. Dermatol.
Volume:	137
Number:	2
Page Range:	S. 332 - 341
Date:	2017
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 HOMOZYGOUS NONSENSE MUTATION; ALPHA-3 CHAIN GENE; LUNG-SPECIFIC LOSS; EPIDERMOLYSIS-BULLOSA; VII COLLAGEN; EXTRACELLULAR-MATRIX; JUNCTIONAL FORMS; MOLECULAR-BASIS; BETA-3 CHAIN; NC1 DOMAIN Multiple languages Dermatology Multiple languages
Refereed:	Yes
URI:	http://kups.ub.uni-koeln.de/id/eprint/24144