Refractory Skin Injury in Complex Knock-out Mice Expressing Only the GM3 Ganglioside

We generated double knock-out mice lacking the GM2/GD2 and the GD3 synthase gene by mating single gene mutants, and we analyzed the abnormal phenotypes of the mutant mice expressing only the GM3 ganglioside. We observed a refractory skin lesion that appeared primarily on the face of the mutant mice...

Full description

Saved in:
Bibliographic Details
Published in:The Journal of biological chemistry 2002-08, Vol.277 (33), p.29881-29888
Main Authors: Inoue, Masahiro, Fujii, Yuko, Furukawa, Keiko, Okada, Masahiko, Okumura, Kenji, Hayakawa, Tetsuo, Furukawa, Koichi, Sugiura, Yasuo
Format: Article
Language:English
Subjects:
Animals
Base Sequence
DNA Primers
Female
G(M3) Ganglioside - genetics
Male
Mice
Mice, Knockout
Microscopy, Electron
N-Acetylgalactosaminyltransferases - genetics
N-Acetylgalactosaminyltransferases - physiology
Sciatic Nerve - pathology
Sialyltransferases - genetics
Sialyltransferases - physiology
Skin - injuries
Skin - ultrastructure
Trigeminal Nerve - pathology
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:We generated double knock-out mice lacking the GM2/GD2 and the GD3 synthase gene by mating single gene mutants, and we analyzed the abnormal phenotypes of the mutant mice expressing only the GM3 ganglioside. We observed a refractory skin lesion that appeared primarily on the face of the mutant mice at 25 weeks after birth or later. Frequent scratching of the wound sites was observed in mutant mice with the skin injury, suggesting that it is a triggering factor that exacerbates the injury. This was confirmed by isolating mice in special cages for metabolic study in which the skin injury developed more rapidly. Characteristic proliferation of nerve fibers was found in the epidermis and subepidermis at the injured sites of the mutants, probably a result of continuous skin injury. Peripheral nerve degeneration was observed in young mutant mice, suggesting that reduced sensory function induced over-scratching and the resulting skin lesion. The fact that sensory response to mechanical stimuli decreased while that to hot stimuli increased in the mutant mice supports this interpretation. Thus, only GM3-expressing mice displayed the important role of gangliosides in maintaining skin integrity via regulation of the peripheral nerves.
ISSN:0021-9258
1083-351X
DOI:10.1074/jbc.M201631200