Hereditary spastic paraplegias (HSPs; SPG1-48) are inherited neurological disorders characterized by lower extremity spasticity and weakness. Loss-of-function mutations in the SPG20 gene encoding spartin cause autosomal recessive Troyer syndrome (SPG20), which has additional features of short stature, cognitive deficits and distal amyotrophy. To identify cellular impairments underlying Troyer syndrome, we ... More
Transient and progressive electrophysiological alterations in the corticostriatal pathway in a mouse model of Huntington's disease.
AuthorsCepeda C, Hurst RS, Calvert CR, Hernández-Echeagaray E, Nguyen OK, Jocoy E, Christian LJ, Ariano MA, Levine MS,
JournalJ Neurosci
PubMed ID12574425
Alterations in the corticostriatal pathway may precede symptomatology and striatal cell death in Huntington's disease (HD) patients. Here we examined spontaneous EPSCs in striatal medium-sized spiny neurons in slices from a mouse model of HD (R6/2). Spontaneous EPSC frequency was similar in young (3-4 weeks) transgenics and controls but decreased ... More
Regenerating motor bridge axons refine connections and synapse on lumbar motoneurons to bypass chronic spinal cord injury.
AuthorsCampos LW, Chakrabarty S, Haque R, Martin JH
JournalJ Comp Neurol
PubMed ID18076081
To restore motor control after spinal cord injury requires reconnecting the brain with spinal motor circuits below the lesion. A bridge around the injury is an important alternative to promoting axon regeneration through the injury. Previously, we reported a novel motor bridge in rats. The thirteenth thoracic nerve was detached ... More