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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)
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)
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)
Thyrotropin-releasing hormone receptor (TRH-R) mRNA was detected in cryostat sections of the mouse testis using biotinylated oligonucleotides complementary to the cDNA encoding the mouse pituitary TRH-R by in situ hybridization. Hybridization signals were detected exclusively in the Leydig cells. The intensity of the signal was probe-concentration(More)
Presumed interneurons are described in the Golgi-impregnated cervical spinal cord taken from normal phenotype and motoneuron-diseased mice of the Wobbler mouse strain (NFR/wr), as well as from the spinal cord of two related normal mouse strains (C57B1/6N and NFR/N). The interneurons, distributed throughout Rexed's laminae V-VIII, are characterized by(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)
Receptor levels for thyrotropin releasing hormone (TRH) measured by quantitative autoradiography in the Wobbler mouse cervical spinal cord show receptor losses that may relate to the inherited loss of motoneurons, most pronounced late (at Stage 4) in the motoneuron disease. An age-related decrease of TRH and serotonin (5-HT) receptors can be seen 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 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)