Abstract: The present invention relates to the engineering an animal model, preferably mammalian models, more preferably a rat model representing Charcot-Marie-Tooth disease 2A (CMT2A) harboring the p.Arg364Trp or p.His361Tyr Mfn2 mutation, whose human counterpart results in a severe, early-onset axonal neuropathy. A model having the p.Arg364Trp Mfn2 mutation is based on a mutation made using zinc finger endonuclease technology in fertilized rat eggs. Cohorts of mutants and wild type littermates were characterized behaviorally and shown to develop multiple motor deficits that worsened over time. Separate cohorts of mutant and wild type rats sacrificed at 7, 40, and 48 weeks and analyzed by light microscopy showed a reduced density of myelinated axons and active axonal degeneration in distal but not proximal nerves, as well as axonal degeneration in the fasciculus gracilis of the cervical spinal cord at 40 and 48 weeks.

Abstract: A self extending storable panel formed of cloth or flexible plastic, with a circular perimeter sleeve, and a spring wire hoop in the sleeve which can be twisted for storage and will extend itself into a hoop in use. Tabs may be attached to the panels for hanging the panels where required.

Abstract: An inertial transformation matrix generator generates a succession of Euler transformation matrices in inertial coordinates, and is useful for converting to inertial coordinates the responses of a sensor hard mounted on the hull of a craft (e.g., an aircraft). First, second and third rate-sensing gyros located proximately to said sensor are strapped down to the craft hull, and are oriented to sense the motion of the craft hull in three mutually orthogonal directions, for providing respective output signals indicative of components of craft hull motion in each of those three mutually orthogonal directions. The output signals of the gyros are digitized, and based on these digital signals successive incremental Euler transformation matrices are generated.