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  • 1
    Electronic Resource
    Electronic Resource
    Alterations in neuron morphology in mucopolysaccharidosis type I (1988)
    Springer
    Acta neuropathologica 75 (1988), S. 611-620 
    Details
    ISSN: 1432-0533
    Keywords: Neuronal storage disease ; Neurite growth ; Dendrite ; Hurler's disease ; Mucopolysaccharidosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Morphological changes in neurons with inborn defects of the lysosomal hydrolase, α-l-iduronidase, and with concomitant storage of glycosaminoglycans, were evaluated by Golgi staining in two animal models and compared to a similar study of a child with the same disease. Cortical pyramidal neurons in feline mucopolysaccharidosis type I often displayed axon hillock enlargements (meganeurites) and/or ectopic, secondary neuritic processes sprouting from this same region of the cell. The latter structures were prominent and often appeared longer than similar neurites reported in other neuronal storage discases. Although most meganeurites were aspiny, a few were observed which possessed spine-like processes or neurites. Other than these morphological changes in cortical pyramidal neurons, few other cell types displayed abnormalities demonstrable by Golgi impregnation. In the canine model of this disorder, abnormal Golgi-impregnated cortical neurons resembled more closely those seen in human mucopolysaccharidosis. That is, they possessed meganeurites which typically were aspiny in appearance. Ectopic neurite growth was not observed on any Golgi-impregnated neurons in the cases of canine or human mucopolysaccharidosis used in this study. The latter finding given the advanced ages of these cases, is consistent with the view that ectopic neuritogenesis seen in neuronal storage diseases may be subject to a developmental window, albeit one open well beyond the period of early postnatal maturation.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00686207
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  • 2
    Electronic Resource
    Electronic Resource
    Growth plate pathology in feline mucopolysaccharidosis VI (1995)
    Springer
    Calcified tissue international 57 (1995), S. 185-190 
    Details
    ISSN: 1432-0827
    Keywords: Dysostosis ; Feline ; Lysosomal ; Mucopolysaccharidosis ; Storage
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine , Physics
    Notes: Abstract The mucopolysaccharidoses (MPS) are a family of lysosomal storage diseases that result from the accumulation of partially catabolized glycosaminoglycans (GAGs) within lysosomes. A characteristic of most affected individuals is radiographic evidence of symmetrical epiphyseal dysplasia, with short stature and degenerative joint disease. Although there is evidence of epiphyseal dysfunction, little is known of the changes that occur at the morphological level. The growth plate of the femoral head was studied by light and electron microscopy in five cats with MPS VI (Maroteaux-Lamy syndrome, arylsulfatase B deficiency) and 12 normal cats. Compared with the normals, the MPS VI cat growth plates exhibited poorly organized proliferative zones, an almost total loss of column formation in the hypertrophic zone, an uneven chondro-osseous junction, a disorganized calcifying cartilage zone, and abnormal or reduced numbers of osteoclasts. By electron microscopy, the cytoplasm of affected cat chondrocytes was filled with membrane-bound vacuoles. Together these findings indicate that the MPS diseases cause major changes in growth plate structure and function.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310256
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    Paper (German National Licenses)
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  • 3
    Electronic Resource
    Electronic Resource
    Inherited XX sex reversal in the cocker spaniel dog (1984)
    Springer
    Human genetics 〈Berlin〉 67 (1984), S. 62-69 
    Details
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Nine XX true hermaphrodites and two XX males were discovered in a family of American cocker spaniels. The true hermaphrodites were partially-masculinized females with ovotestes; the XX males had malformed male external genitalia and cryptorchid aspermatogenic testes. Wolffian and Mullerian duct derivatives were present in both true hermaphrodites and XX males. All four sires of sex-reversed dogs were normal XY males; five of the dams were anatomically normal females and one was an XX true hermaphrodite. A second true hermaphrodite reproduced as a female, producing anatomically normal offspring. All matings that produced sex-reversed offspring were consanguineous. Matings of the parents of sex-reversed cocker spaniels to normal beagles with no family history of intersexuality produced only normal offspring. Examination of G-banded karyotypes of the affected animals, their parents, and siblings, revealed no structural anomalies of the chromosomes that were consistently associated with sex-reversal. In assays for serologically-detectable H-Y antigen, the group of XX true hermaphrodites and the group of XX males had mean levels of the antigen not significantly different from that in normal male controls. Female parents of sex-reversed dogs and some of their female siblings were typed H-Y antigen positive, but the mean level of the antigen in this group was less than that of normal male controls. It is proposed that XX sex reversal in cocker spaniels is due to a mutant gene which when homozygous in females, results in a level of H-Y antigen similar to that found in normal males and the gonads develop as ovotestes or testes. When the gene is heterozygous in females, the level of serologically-detectable H-Y antigen is lowr than that found in normal males and the gonads develop as normal ovaries. The persistence of Mullerian structures in the presence of testicular tissue suggests that Mullerian inhibiting substance is deficient or ineffective in its action in this condition.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00270560
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    Paper (German National Licenses)
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