Characteristics of hydrocephalus expression in the LEW/Jms rat strain with inherited disease

Abstract

Materials and methods:The expression and severity of hydrocephalus was characterized in LEW/Jms rats with inherited hydrocephalus. Results: 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 × 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%. Conclusion: 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.

Clinical and neuropathological evolution of the hydrocephalus developed by the mutant mouse hyh

“…factors may influence the degree of severity of this pathology. This is in accordance with findings in other hydrocephalic mutant strains [3,4]. The present investigation was designed to (a) study the clinical evolution of hydrocephalic mice in order to evaluate wether or not clinical heterogeneity does actually occur, (b) identify nongenetic factors (maternal age, multiparity) that may affect such an evolution, and (c) identify neuropathologic events underlying clinical heterogeneity.…”

Understanding and Modeling the Pathophysiology of Hydrocephalus: In Search of Better Treatment Options

“…LEW/Jms rats have a mutation that leads to the development of hydrocephalus immediately after birth [70]. It was noticed that more male than female rats developed symptoms, so the genetic cause may be sex-linked [70]. This model could be used to study human diseases like X-linked hydrocephalus.…”

Understanding and Modeling the Pathophysiology of Hydrocephalus: In Search of Better Treatment Options

“…LEW/Jms rats have a mutation that leads to the development of hydrocephalus immediately after birth [58]. It was noticed that more male than female rats developed symptoms, so the genetic cause may be sex-linked [58]. This model could be used to study human diseases like X-linked hydrocephalus.…”