Wild-type and mutant SOD1 share an aberrant conformation and a common pathogenic pathway in ALS.

Many mutations confer one or more toxic function(s) on copper/zinc superoxide dismutase 1 (SOD1) that impair motor neuron viability and cause familial amyotrophic lateral sclerosis (FALS). Using a conformation-specific antibody that detects misfolded SOD1 (C4F6), we found that oxidized wild-type SOD1 and mutant SOD1 share a conformational epitope that is not present in normal wild-type SOD1. In a subset of human sporadic ALS (SALS) cases, motor neurons in the lumbosacral spinal cord were markedly C4F6 immunoreactive, indicating that an aberrant wild-type SOD1 species was present.

Preliminary evaluation of a unique freezing technology for bovine spermatozoa cryopreservation.

Artificial insemination (AI) and semen cryopreservation has significantly improved the breeding potential of male animals. However, current freezing techniques commonly result in reduced semen quality. Ten years ago, a unique freezing technology (UFT) was developed for the freezing of foodstuffs and other materials.

Preliminary trial: motility comparisons of a unique freezing technology (UFT) to liquid nitrogen mist methodology for cryopreservation of porcine spermatozoa.

The motility outcomes of boar semen frozen with newly developed freezing techniques using a new unique freezing technology (UFT) compared with traditional liquid nitrogen methodology were investigated with the intent of improving current fertility outcomes using semen. The UFT is an electronically controlled cooling chamber that houses an organic fluid bath that can be maintained at temperatures below 0 degrees C without solidifying to freeze samples. Four ejaculates from four different boars were collected for this trial.

Disruption of muscle membrane and phenotype divergence in two novel mouse models of dysferlin deficiency.

Limb girdle muscular dystrophy type 2B and Miyoshi myopathy are clinically distinct forms of muscular dystrophy that arise from defects in the dysferlin gene. Here, we report two novel lines of dysferlin-deficient mice obtained by (a) gene targeting and (b) identification of an inbred strain, A/J, bearing a retrotransposon insertion in the dysferlin gene. The mutations in these mice were located at the 3' and 5' ends of the dysferlin gene.

Multiple myeloma presenting as space-occupying lesions of the liver.

Initial myeloma presentation as discrete liver nodules is unusual. A 59-year-old man who presented with hepatomegaly and multiple space-occupying lesions in the liver and who was found to have a lambda light chain multiple myeloma with hepatic plasmacytomas is described and a review of liver involvement in multiple myeloma is presented.