Survival and lung pathology of @entity19 models of XXXX and @entity13277 .

@entity1 @entity71107 @entity19 @entity735 @entity358 @entity74 @entity13276 @entity13277 @entity6353
@entity13276 ( @entity13276 ), a @entity74 in @entity1 , affects the biosynthesis/processing of the related intracellular organelles: lysosomes, melanosomes, and platelet dense granules. The disease is multigenic in both @entity1 and @entity19 where 14 separate genes have been demonstrated to be causative. @entity1 often die prematurely with @entity358 . @entity1 with the related @entity13277 ( @entity13277 ) likewise have significantly reduced life spans. Long-term survival and lung histomorphology were analyzed in a pilot experiment involving several genetically defined singly and doubly mutant @entity19 @entity13276 mutants and the beige @entity13277 mutant to determine whether these parameters are altered in the @entity19 models. The mutants differed widely in both longevity and lung architecture. @entity19 doubly homozygous for the @entity735 or for the @entity71107 and pearl genes had the shortest life spans with none surviving the two-year experimental duration. Life spans were similarly severely reduced in the beige and gunmetal mutants. Intermediate life spans were apparent in the pearl, pallid, and @entity6353 mutants whereas minimal effects were noted in ruby eye, @entity71107 , light ear, and @entity6353 mutants. Enlarged air spaces were noted in histologic sections of lungs of several of the mutants. For the most part, the severity of @entity358 was inversely proportional to the long-term survival of these various mutants, suggesting that lung pathology may contribute to mortality, as has been suggested for @entity1 @entity13276 @entity1 .
