Asebia-2J (Scd1(ab2J)): a new allele and a model for scarring alopecia.
A spontaneous, autosomal, recessive mouse mutation exhibiting mild scaly skin, progressive scarring alopecia, slightly runted growth, and photophobia arose at The Jackson Laboratory in 1993 in the inbred mouse strain DBA/1LacJ. Because this mutant mouse showed genetic, anatomical, and laboratory similarities to the asebia mutation, crosses were done between the new mutant and mice carrying the asebia-J allele. Because the F1 offspring were affected, indicating the two mutants were allelic, the new mutation was named asebia-2J. Careful histological analysis of skin development of mice homozygous and heterozygous for either asebia-J or asebia-2J revealed that both types of mutant mice are very similar regardless of their background. Notable histopathological features of mice homozygous for either allele included extreme sebaceous gland hypoplasia, abnormally long anagen follicles, retained inner root sheath, hair fiber perforation of the anagen follicle base, and progressive follicular replacement by scarring. In this article we present a new pathogenetic hypothesis based on the importance of the sebaceous gland in hair fiber sheath dissociation: in the absence of a functional sebaceous gland the hair follicle is destroyed. The cutaneous pathology of this mutant mouse underscores the importance of the sebaceous gland to follicular biology and presents an animal model for studying the human scarring alopecias, which characteristically begin with sebaceous gland ablation.
Sundberg
J P
JP
Jackson Laboratory, Bar Harbor, Maine 04609-1500, USA. jps@jax.org
Boggess
D
D
Sundberg
B A
BA
Eilertsen
K
K
Parimoo
S
S
Filippi
M
M
Stenn
K
K
D000483:Alleles; D000505:Alopecia / Q000235:genetics* / Q000473:pathology; D000822:Animal Husbandry; D000818:Animals; D002921:Cicatrix / Q000235:genetics* / Q000473:pathology; D004817:Epidermis / Q000378:metabolism; D006579:Heterozygote; D006720:Homozygote; D051379:Mice; D008811:Mice, Inbred DBA / Q000235:genetics; D008855:Microscopy, Electron, Scanning; D009154:Mutation; D012867:Skin / Q000473:pathology; D014870:Water Loss, Insensible