Panteleyev, 2000  Title: Ornithine decarboxylase transgenic mice
as a model for human atrichia with papular lesions.
Authors: Panteleyev AA, Christiano AM, O'Brien
TG, Sundberg JP
Journal: Exp Dermatol 2000 Apr;9(2):146-51
PMID: 10772389, UI: 20233343
Affiliated institution: Department
of Dermatology, Columbia University, New York, NY, USA.
Cited in:
The hair follicle is characterized by cyclic transformations
from active growth and hair fiber production through regression
into a resting phase. The growth phase, known as anagen, is associated
with rapid rates of cell turnover, and variations in the rate
of DNA synthesis in mouse skin throughout the hair cycle are accompanied
by changes in the activity of ornithine decarboxylase (ODC), a
key enzyme in the synthesis of polyamines, which are actively
involved in regulation of normal cell division, differentiation,
and growth. Previously, a transgenic mouse was created that overexpressed
ODC in the skin using a K6 promoter. The first hair cycle in neonatal
transgenic mice appeared to be normal, but by the third week of
postnatal life transgenic pups begin to progressively lose hair.
The lower portion of the hair follicle was progressively replaced
with enlarging cystic structures located in the deep dermis, and
the transgenic mice exhibited excessive growth of skin mass resulting
in pronounced wrinkling and folding. Interestingly, these findings
bore striking resemblance to the rhino mouse phenotype and to
human patients with papular atrichia, a rare congenital ectodermal
disorder characterized by progressive and irreversible hair loss
in early childhood. The similarities in phenotype between transgenic
mice and human atrichia with papular lesions suggest that ODC
transgenics may represent a useful model for studying this disorder.
It appears that ODC plays a functionally important, yet still
obscure role in a complex metabolic pathway that is critical in
hair follicle function not only in mice, but in humans as well.
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