Aoyama H, Fujii S, Teramoto S, Shirasu Y
Mitsukaido Laboratories, Institute of Environmental Toxicology, Ibaraki, Japan.
J Hered. 1991 Sep-Oct;82(5):397-401. doi: 10.1093/oxfordjournals.jhered.a111110.
A new mutant gene that causes preaxial polydactyly in the hindlimbs was found in the strain of rats with fused pulmonary lobes (fpl). Genetic analysis has revealed that the new mutation is inherited as an autosomal recessive trait and is not closely linked with the fpl gene. Since homozygous mutants die within the first 2 days after birth, the mutant gene was named polydactyly lethal, gene symbol pl. A test for allelism between the pl gene and another gene, pd, which also causes preaxial duplication anomalies, showed no allelism between these two genes. Skeletal examination revealed that all pl/pl newborns had thickening and/or bifurcation of tarsal I and metatarsal I, as well as duplication of the proximal and distal phalanges of digit I in the hindlimbs. In some cases, phalangeal duplication or bifurcation in digit I with thickening of metacarpal I was also found in the forelimbs, although extra forelimb digits were not detected externally. The pl/pl newborns showed hunchback-like abnormal posture externally and had several associated vertebral abnormalities in varying degrees, i.e., kyphosis, scoliosis, splitting of the thoracic vertebral bodies, and fusion of the lumbar vertebral bodies. No major malformations were seen in the visceral organs. The cause of neonatal deaths has not yet been determined.
在肺叶融合(fpl)大鼠品系中发现了一个导致后肢轴前多指畸形的新突变基因。遗传分析表明,该新突变以常染色体隐性性状遗传,且与fpl基因没有紧密连锁。由于纯合突变体在出生后的头两天内死亡,该突变基因被命名为多指致死基因,基因符号为pl。对pl基因与另一个也会导致轴前重复异常的基因pd进行等位性测试,结果显示这两个基因之间不存在等位性。骨骼检查发现,所有pl/pl新生大鼠的跗骨I和跖骨I都有增厚和/或分叉,以及后肢第一趾的近端和远端指骨重复。在某些情况下,前肢也发现了第一指的指骨重复或分叉以及第一掌骨增厚,不过在外部未检测到额外的前肢趾。pl/pl新生大鼠外部表现出驼背样异常姿势,并且有不同程度的几种相关脊柱异常,即脊柱后凸、脊柱侧凸、胸椎椎体分裂和腰椎椎体融合。在内脏器官中未发现重大畸形。新生儿死亡的原因尚未确定。