Jones Hazel C, Carter Barbara J, Morel Laurence
Department of Pharmacology and Therapeutics, University of Florida, P.O. Box 100267, Gainesville, FL 32610, USA.
Childs Nerv Syst. 2003 Jan;19(1):11-8. doi: 10.1007/s00381-002-0671-3. Epub 2002 Dec 13.
The expression and severity of hydrocephalus was characterized in LEW/Jms rats with inherited hydrocephalus.
The frequency of overt disease (lethal) was 27.7%, but varied depending on breeding line. It increased to 31.5% when rats with mild disease were included. Most breeding pairs (48/51) produced hydrocephalic offspring with significantly more males than females affected. Some adult rats were also found to have mild (nonlethal) ventricular dilatation. When bred to Fisher F344 rats, 3% of the F(1) progeny had overt hydrocephalus, indicating that the trait is not recessive. Overt hydrocephalus was 9-20% in N(2) rats (F(1) rats x LEW/Jms). The frequency of hydrocephalus and the presence of an excess of hydrocephalic males, varied depending on the direction of the cross. Mild hydrocephalus in N(2) rats was 3.1%.
It is concluded that the inheritance of hydrocephalus in LEW/Jms strain is probably not mendelian recessive but may be semidominant or involve more than one gene and has a male bias indicating possible linkage to sex chromosomes.
在患有遗传性脑积水的LEW/Jms大鼠中对脑积水的表达和严重程度进行了表征。
显性疾病(致死性)的发生率为27.7%,但因繁殖系而异。当纳入患有轻度疾病的大鼠时,该发生率增至31.5%。大多数繁殖对(48/51)产生的脑积水后代中,受影响的雄性明显多于雌性。还发现一些成年大鼠有轻度(非致死性)脑室扩张。当与Fisher F344大鼠杂交时,3%的F(1)后代有显性脑积水,表明该性状不是隐性的。N(2)大鼠(F(1)大鼠×LEW/Jms)中的显性脑积水为9 - 20%。脑积水的发生率以及脑积水雄性过多的情况因杂交方向而异。N(2)大鼠中的轻度脑积水为3.1%。
得出的结论是,LEW/Jms品系中脑积水的遗传可能不是孟德尔隐性遗传,而可能是半显性遗传或涉及多个基因,并且存在雄性偏倚,表明可能与性染色体连锁。