BSSG rat

In vivo models of Parkinson’s disease – Mammalian models

TOXIC MODELS

BSSG

During the 1940’s the indigeneous Chamaroo people of the island of Guam lived on a diet that consisted largely of flour made from washed seeds of the plant Cycas micronesia (cycad). The  consumption of this flour has been suggested as an underlying cause of the amyotrophic lateral sclerosis(ALS)/parkinsonism-dementia complex (ALS-PDC) that emerged in the island starting in 1953 (25% of adult death were due to ALS-PDC).

Wash cycad flour contains a number of water-insoluble neutoxic molecules, the most toxic being beta-sitosterol beta-D-glucoside (BSSG). The compound is toxic in a variety of cellular models and in organotypic slices. In mice consumption of BSSG lead to a progressive development on an ALS-PDC phenotype with a loss of motor neuron in the spinal cord. In rats, BSSG induces a progressive neurodegeneration that presents a parkinsonsim phenotype, with no evidence of spinal motor neuron pathology.