The levels of cyclic GMP and glucose 1,6-diphosphate,and the activity of phosphofructokinase,in muscle from normal and dystrophic mice

A striking reduction in the levels of glucose 1,6-diphosphate and an increase in cyclic GMP were found in muscle from dystrophic mice. Concomitant to these changes, the allosteric activity of phosphofructokinase was found to be markedly reduced. These findings could offer an explanation for the observed reduction in glycolysis in the dystrophic muscle.     

Animal models for Gaucher disease research

Gaucher disease (GD), the most common lysosomal storage disorder (LSD), is caused by the defective activity of the lysosomal hydrolase glucocerebrosidase, which is encoded by the GBA gene. Generation of animal models that faithfully recapitulate the three clinical subtypes of GD has proved to be more of a challenge than first anticipated. The first mouse to be produced died within hours after birth owing to skin permeability problems, and mice with point mutations in Gba did not display symptoms correlating with human disease and also died soon after birth. Recently, conditional knockout mice that mimic some features of the human disease have become available. Here, we review the contribution of all currently available animal models to examining pathological pathways underlying GD and to testing the efficacy of new treatment modalities, and propose a number of criteria for the generation of more appropriate animal models of GD.     

The giant cells produced by Telenomus remus (Hymenoptera: Scelionidae)

The parasitic larva of Telenomus remus is surrounded by giant cells throughout its first instar. These cells arise in the embryonic serosa of the parasite and grow in size, starting with a radius of 5nm and ending with 27nm. Young cells are round and mononuclear, whereas older ones are often polynuclear and have varied, irregular contours. Most cells are profusely vacuolated, the vacuoles being especially large in some of the older cells. Only a few of the giant cells are devoured by the first instar parasite larva, but all disappear at the end of this stage. No giant cells seem to be produced by supernumerary larvae. Once the parasite egg hatches, the host tissue disintegrates almost instantaneously.