Neonatal growth delay in alpha-1-antitrypsin disease. Influence of genetic background.
We have developed a transgenic mouse strain, Z#2, which represents a model for alpha 1-protease inhibitor (alpha 1-antitrypsin: alpha 1-Pi)-associated liver disease (Dycaico et al., 1988). Fifteen percent of human infants with alpha 1-Pi disease develop non-viral hepatitis which is sometimes associated with growth retardation. Such hepatitis and growth retardation tend to occur in a subset of families with other alpha 1-Pi affected members who have had non-viral hepatitis. The Z#2 mouse strain exhibits non-viral hepatitis and growth retardation. This phenotype is more pronounced in transgenic offspring of crosses between Z#2 mice and DBA/2J inbred mice, and less pronounced in transgenic offspring of crosses between Z#2 and CBA/J inbred mice. Such phenotypic differences resemble the phenotypic differences seen in human families with alpha 1-Pi-associated liver disease.
Dycaico
M J
MJ
Stratagene, La Jolla, CA 92037.
Felts
K
K
Nichols
S W
SW
Geller
S A
SA
Sorge
J A
JA
D000818:Animals; D000831:Animals, Newborn; D001835:Body Weight; D003433:Crosses, Genetic; D005260:Female; D005317:Fetal Growth Retardation / Q000235:genetics*; D008297:Male; D051379:Mice; D008822:Mice, Transgenic; D010641:Phenotype; D011247:Pregnancy; D019896:alpha 1-Antitrypsin Deficiency