Ll Vacca-Galloway

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The Wobbler mouse (wr) has been proposed as a model for human inherited motoneuron disease (infantile spinal muscular atrophy). The primary defect is thought to be in the motoneurons. Therefore we undertook a survey of the qualitative and quantitative changes occurring in the cervical spinal motoneurons of Wobbler mice during a late stage of the motoneuron(More)
Previous pathological reports have indicated that swollen and vacuolated motoneuron cell bodies are the most predominant feature characterising Wobbler mouse motoneuron disease, but there has been little supportive evidence using area measurements. The present study focuses on the possible role of changes in neuronal nuclear and perikaryal volumes in the(More)
The Wobbler mouse possesses an inherited motoneuron disease, which expresses itself primarily at cervical spinal levels and in cranial motor nuclei. Cell degeneration is sporatic and negligible in other motor regions of the brain (e.g., cerebellum, corpus striatum). However, enkephalin concentrations are consistently lower in the Wobbler cerebellum(More)
The Wobbler mouse possesses an inherited autosomal recessive form of motoneuron disease. The most characteristic abnormality is the degeneration of motoneurons, mostly in the cervical spinal cord, and in the brain stem cranial motor nuclei. The underlying pathology shows up as symptoms that are only detectable confidently around the time of weaning (age 3(More)
The mutant mouse, wobbler, possesses a recessively inherited degeneration of motoneurons and other ventral horn cells in the cervical spinal cord, and therefore it has been proposed as an animal model of human motoneuron disease. Affected mice have been identified by behavioral tests that also determined the extent of the motor deficit. The results from(More)
Using immunocytochemistry (ICC), the number of immunoreaction products (IRPs) visualized for the peptide substance P (SP) appears reduced within the human spinal cord (also substantia nigra) taken at autopsy from cases diagnosed with Huntington's disease (HD) compared with the non-HD cases (Vacca 1983). The reductions of SR-IRPs become apparent in the HD(More)
The present study was undertaken to quantify selected neuropeptides (thyrotropin releasing hormone, substance P, methionine and leucine enkephalin) in the cervical spinal cord and other regions of the central nervous system of Wobbler mice by radioimmunoassays during several stages of the motoneuron disease compared with age- and sex-matched normal(More)
The Wobbler mouse possesses an inherited form of motoneuron disease that expresses itself most dramatically in the forelimbs. Previous immunocytochemical (ICC) studies have shown that neuronal processes containing substance P (SP), thyrotropin releasing hormone (TRH) and serotonin (5-HT) seem to sprout in the ventral horn of the cervical spinal cord taken(More)
The Wobbler mouse is used as a model of human motoneuron disease (MND). During the disease progress, the significant loss of motoneurons in cervical spinal cord and cranial motor nuclei leads to the progressive loss of motor function in the forelimb, head, and neck regions. The loss of cutting and chewing ability that results in the inability to feed(More)
The Wobbler mouse (wr) exhibits the loss of motoneurons especially in the cervical spinal cord, and thus has been studied as a model for human motoneuron diseases. Wobbler mice selected at various ages and stages during the disease process show increased levels of thyrotropin releasing hormone and substance P in spinal cord and brainstem (medulla).(More)