GM1 gangliosidosis in friesian calves

Donnelly, W. J. C.; Sheahan, B. J.; Rogers, Terence A.

doi:10.1002/path.1711110305

Cited by 44 publications

(13 citation statements)

References 18 publications

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“…23,24 Dysmyelinogenesis in GM 1 -gangliosidosis of ESS and PWD was characterized by loss of myelin, reduced amounts of cerebrosides and sulfatides in the gray and white matter, and increased amounts of stage-specific embryonic antigen 1 glycolipid in the gray matter. 23,24 A paucity of oligodendrocytes associated with myelin deficiency oc-curred also in cats 12 and Friesian cattle 17 with GM 1 -gangliosidosis. Myelin rarefication has also been described in two domestic shorthair cats and one Siamese cat.…”

Section: Discussionmentioning

confidence: 99%

“…Corresponding age-, size-, and sex-matched dogs (Nos. [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19] were used as controls. Samples of various tissues, including brain, spinal cord, eye, liver, kidney, urinary bladder, spleen, lymph nodes, lung, heart, intestine, pancreas, salivary gland, adrenal gland, thyroid gland, bones, bone marrow, and skin, were collected during necropsy and fixed in 10% formalin.…”

Section: Light and Electron Microscopymentioning

confidence: 99%

“…1-3 and controls (Nos. [10][11][12][13][14][15][16][17][18][19] were incubated with primary antibodies directed against glial fibrillary acidic protein (GFAP), myelin basic protein (MBP), neurofilament (NF), and major histocompatibility complex class II (MHC II) antigen employing the avidin-biotin-peroxidase complex method as previously described. 10,18,21 Sections (3 m thick) were deparaffinized in xylene and rehydrated through graded alcohols.…”

Section: Immunohistochemistrymentioning

confidence: 99%

“…38 The disease has been described in a variety of species, including cats, cattle, sheep, mice, and humans. [1][2][3][4][5][6][7][8][9]12,14,16,17,[27][28][29]31,[33][34][35][36][37][38] Until now, the canine form has been found in English Springer Spaniels (ESS), 3,33 Portuguese Water Dogs (PWD), 3,35,36 mixed-breed Beagles, 34 and Alaskan Huskies. 28 In humans, the disorder is classified as infantile (type 1), juvenile (type 2), and adult (type 3) forms.…”

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confidence: 99%

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GM₁-gangliosidosis in Alaskan Huskies: Clinical and Pathologic Findings

et al. 2001

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Abstract. Three Alaskan Huskies, two females and one male, were diagnosed with GM 1 -gangliosidosis. Clinically, diseased animals exhibited proportional dwarfism and developed progressive neurologic impairment with signs of cerebellar dysfunction at the age of 5-7 months. Skeletal lesions characterized by retarded enchondral ossification of vertebral epiphyses were revealed by radiographs of the male dog at 5.5 months of age. Histologic examination of the central nervous system (CNS) revealed that most neurons were enlarged with a foamy to granular cytoplasm due to tightly packed vacuoles that displaced the Nissl substance. Vacuoles in paraffin-embedded sections stained positively with Luxol fast blue and Grocott's method, and in frozen sections vacuoles were periodic acid-Schiff positive. Foamy vacuolation also occurred within neurons of the autonomic ganglia. Extracerebral cells such as macrophages and peripheral lymphocytes also displayed foamy cytoplasm and vacuolation. In the CNS of diseased animals, a mild demyelination and axonal degeneration was accompanied by a significant astrogliosis (P Ͻ 0.05) in the gray matter as compared with age-and sex-matched control dogs. There was also a significant loss (P Ͻ 0.05) of oligodendrocytes in the gray and white matter of affected animals as compared with controls. Ultrastructurally, the neuronal storage material consisted of numerous circular to concentric whorls of lamellated membranes or stacks of membranes in parallel arrays. GM 1 -gangliosidosis in Alaskan Huskies resembles ␤-galactosidase deficiency in other canine breeds, and these CNS disorders may be a consequence of neuronal storage and disturbed myelin processing.

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Section: Discussionmentioning

confidence: 99%

Section: Light and Electron Microscopymentioning

confidence: 99%

Section: Immunohistochemistrymentioning

confidence: 99%

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confidence: 99%

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GM₁-gangliosidosis in Alaskan Huskies: Clinical and Pathologic Findings

et al. 2001

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“…The relative proportions in diseased tissues of the three major ganglicsides, designated GN1, GDla , ~ud CT, differ from those in normal cerebrum. Whereas normal cerebrums contain 30 to 40% GN 1 ganglioside, the cerebrums of affected calves contain 60 to 80% of this component with a corresponding 50% reduction in gangliosides GDlaSJ~d GT (Donnelly et al, 1973b). Differences in the distribution of ganglioside species are obvious on thin layer c]u'omato,j~ar.~s (Donnelly ~nd She~1~1, 1975).…”

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confidence: 95%