The Experts below are selected from a list of 87 Experts worldwide ranked by ideXlab platform

Bruce L Homer - One of the best experts on this subject based on the ideXlab platform.

  • naturally occurring gm2 gangliosidosis in two muntjak deer with pathological and biochemical features of human classical tay sachs disease type b gm2 gangliosidosis
    Acta Neuropathologica, 1999
    Co-Authors: Y T Li, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, Bruce L Homer
    Abstract:

    Two juvenile sibling male Muntjak deer (Muntiacus muntjak) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of GM2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B GM2 gangliosidosis (classical Tay-Sachs disease).

B. Homer - One of the best experts on this subject based on the ideXlab platform.

  • Naturally occurring G_M2 gangliosidosis in two Muntjak deer with pathological and biochemical features of human classical Tay-Sachs disease (type B G_M2 gangliosidosis)
    Acta Neuropathologica, 1999
    Co-Authors: J. Fox, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, B. Homer
    Abstract:

    Two juvenile sibling male Muntjak deer ( Muntiacus muntjak ) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of G_M2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B G_M2 gangliosidosis (classical Tay-Sachs disease).

Glyn Dawson - One of the best experts on this subject based on the ideXlab platform.

  • naturally occurring gm2 gangliosidosis in two muntjak deer with pathological and biochemical features of human classical tay sachs disease type b gm2 gangliosidosis
    Acta Neuropathologica, 1999
    Co-Authors: Y T Li, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, Bruce L Homer
    Abstract:

    Two juvenile sibling male Muntjak deer (Muntiacus muntjak) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of GM2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B GM2 gangliosidosis (classical Tay-Sachs disease).

  • Naturally occurring G_M2 gangliosidosis in two Muntjak deer with pathological and biochemical features of human classical Tay-Sachs disease (type B G_M2 gangliosidosis)
    Acta Neuropathologica, 1999
    Co-Authors: J. Fox, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, B. Homer
    Abstract:

    Two juvenile sibling male Muntjak deer ( Muntiacus muntjak ) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of G_M2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B G_M2 gangliosidosis (classical Tay-Sachs disease).

A Alleman - One of the best experts on this subject based on the ideXlab platform.

  • naturally occurring gm2 gangliosidosis in two muntjak deer with pathological and biochemical features of human classical tay sachs disease type b gm2 gangliosidosis
    Acta Neuropathologica, 1999
    Co-Authors: Y T Li, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, Bruce L Homer
    Abstract:

    Two juvenile sibling male Muntjak deer (Muntiacus muntjak) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of GM2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B GM2 gangliosidosis (classical Tay-Sachs disease).

  • Naturally occurring G_M2 gangliosidosis in two Muntjak deer with pathological and biochemical features of human classical Tay-Sachs disease (type B G_M2 gangliosidosis)
    Acta Neuropathologica, 1999
    Co-Authors: J. Fox, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, B. Homer
    Abstract:

    Two juvenile sibling male Muntjak deer ( Muntiacus muntjak ) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of G_M2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B G_M2 gangliosidosis (classical Tay-Sachs disease).

J Johnsrude - One of the best experts on this subject based on the ideXlab platform.

  • naturally occurring gm2 gangliosidosis in two muntjak deer with pathological and biochemical features of human classical tay sachs disease type b gm2 gangliosidosis
    Acta Neuropathologica, 1999
    Co-Authors: Y T Li, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, Bruce L Homer
    Abstract:

    Two juvenile sibling male Muntjak deer (Muntiacus muntjak) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of GM2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B GM2 gangliosidosis (classical Tay-Sachs disease).

  • Naturally occurring G_M2 gangliosidosis in two Muntjak deer with pathological and biochemical features of human classical Tay-Sachs disease (type B G_M2 gangliosidosis)
    Acta Neuropathologica, 1999
    Co-Authors: J. Fox, Glyn Dawson, A Alleman, J Johnsrude, Juergen Schumacher, B. Homer
    Abstract:

    Two juvenile sibling male Muntjak deer ( Muntiacus muntjak ) with histories of depression, ataxia, circling and visual deficits were studied. Cerebrospinal fluid analyses revealed vacuolated macrophages that contained long parallel needle-like intracytoplasmic inclusions. Light microscopically, nerve cell bodies throughout the Brain, Ganglion cells within the retina and neurons in the myenteric plexuses were variably swollen and had pale granular to finely vacuolated eosinophilic cytoplasm. Neuronal cytoplasm stained specifically with sudan black and Luxol-fast blue stains. Within the Brain there were occasional axonal spheroids, foci of astrogliosis and scattered microglial cells with abundant pale foamy cytoplasm. Electron microscopy of the Brain and retina revealed numerous neurons and Ganglion cells, respectively, with multiple membrane-bound structures that contained compact electron-dense membranous whorls and fewer parallel membranous stacks. Thin layer chromatography of total lipid extracts of the cerebral cortex of both cases revealed massive accumulation of G_M2 ganglioside. Crude kidney extracts of the two affected deer were able to hydrolyze 4-methylumbelliferyl β-GlcNAc, but not 4-methylumbelliferyl β-GlcNAc-6-sulfate, indicating the defect of β-hexosaminidase A. Cellogel electrophoresis of the kidney extracts also revealed the deficiency of β-hexosaminidase A in the two deer. It is concluded that these two deer had the biochemical lesion identical to that of human type B G_M2 gangliosidosis (classical Tay-Sachs disease).