The Fraser Complex Proteins (Frem1, Frem2, and Fras1) Can Form Anchoring Cords in the Absence of AMACO at the Dermal-Epidermal Junction of Mouse Skin

AMACO (VWA2 protein), secreted by epithelial cells, is strongly expressed at basement membranes when budding or invagination occurs in embryos. In skin, AMACO associates with proteins of the Fraser complex, which form anchoring cords. These, during development, temporally stabilize the dermal-epider...

Full description

Saved in:
Bibliographic Details
Published in:International journal of molecular sciences 2023-04, Vol.24 (7), p.6782
Main Authors: Esho, Temitope, Kobbe, Birgit, Tufa, Sara F, Keene, Douglas R, Paulsson, Mats, Wagener, Raimund
Format: Article
Language:English
Subjects:
AMACO
anchoring cords
Animals
basement membrane
Basement membranes
Basements
Blistering
Collagen
Colorectal cancer
Cords
Embryonic development
Embryos
Epithelial cells
Epithelial Cells - metabolism
Epithelium
extracellular matrix
Extracellular Matrix - metabolism
Extracellular Matrix Proteins - metabolism
Fibrils
Fraser complex proteins
Fraser Syndrome - metabolism
Genotype & phenotype
Hair
Humans
immunogold electron microscopy
Kidneys
Lungs
Membranes
Mice
Microscopy
Phenotypes
Proteins
Skin
Skin - metabolism
Stem cells
Syndactyly
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:AMACO (VWA2 protein), secreted by epithelial cells, is strongly expressed at basement membranes when budding or invagination occurs in embryos. In skin, AMACO associates with proteins of the Fraser complex, which form anchoring cords. These, during development, temporally stabilize the dermal-epidermal junction, pending the formation of collagen VII-containing anchoring fibrils. Fraser syndrome in humans results if any of the core members of the Fraser complex (Fras1, Frem1, Frem2) are mutated. Fraser syndrome is characterized by subepidermal blistering, cryptophthalmos, and syndactyly. In an attempt to determine AMACO function, we generated and characterized AMACO-deficient mice. In contrast to Fraser complex mutant mice, AMACO-deficient animals lack an obvious phenotype. The mutually interdependent basement membrane deposition of the Fraser complex proteins, and the formation of anchoring cords, are not affected. Furthermore, hair follicle development in newborn AMACO-deficient mice showed no gross aberration. Surprisingly, it appears that, while AMACO is a component of the anchoring cords, it is not essential for their formation or function.
ISSN:1422-0067
1661-6596
1422-0067
DOI:10.3390/ijms24076782