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  • Ubiquitin  (3)
  • Fluid-phase endocytosis  (2)
  • Immunocytochemistry  (2)
  • Phodopus sungorus  (2)
  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 86 (1993), S. 105-108 
    ISSN: 1432-0533
    Keywords: Pallido-luysio-nigral atrophy ; Motor neuron disease ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A case of motor neuron disease (MND) with pallido-luysio-nigral atrophy (PLNA) is reported. The 45-year-old male patient presented with lower motor neuron symptoms and signs of basal ganglia disturbance. He died after a progressive course of 7 months. Neuropathological examination revealed motor neuron loss at all spinal cord levels with sparing of Onuf's nucleus. Nerve cell loss and gliosis were also present in substantia nigra, globus pallidus, and subthalamic nucleus. The presence of ubiquitin-positive inclusions, a hallmark of most variants of MND, confirms this case as an example of MND. At immunoelectron microscopy the granules were distributed on filamentous material. The combination of clinically apparent PLNA with MND has only been described twice previously. The relationship of this syndrome to other forms of MND and its nosological placement are discussed.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Infantile multiple system atrophy ; Ubiquitin ; Neuronal intraunclear hyaline inclusion disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report presents a case of infantile multiple system atrophy with probably autosomal recessive inheritance. The female patient developed generalized muscular hypotonia, myoclonias and tonic-clonic seizures at the age of 8 months, followed by gradual development of choreoathetotic hyperkinesia and increasing psychomotor retardation. Metabolic disease was ruled out and the child died of aspiration pneumonia at the age of 5 years. General autopsy was unremarkable, but neuropathological examination showed degeneration of cerebellum, inferior olives, medial thalamus, Clarke's nucleus, anterior horn cells, corticospinal, spinocerebellar tracts, and posterior columns. Immunohistochemically many neurons contained intranuclear and intracytoplasmic ubiquitin-positive inclusions, which did not contain neurofilament or tau epitopes and ultrastructurally consisted of granulofilamentous material. We tentatively classify this case as a form of infantile multiple system atrophy linked to neuronal intranuclear hyaline inclusion disease.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Child's nervous system 11 (1995), S. 610-615 
    ISSN: 1433-0350
    Keywords: Nemaline myopathy ; Cardiac involvement ; Nervous system involvement ; Ubiquitin
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Nemaline myopathy belongs to the group of congenital non-progressive myopathies; however, in rare cases death occurs in early infancy. We report two cases of rapidly fatal nemaline myopathy. The first patient, who died at the age of 26 months, showed atrophy of type 1 fibers containing numerous rods in biopsy sections. Biopsy of the second patient, who had died at the age of 5 months, revealed severe maturational arrest and myopathy, but rods were so rare that diagnosis could only be made at the ultrastructural level. Autopsy of both patients showed that atrophy of type 1 fibers and maturational arrest had disappeared in the very same muscles; rods had moved to a central position in the first and significantly increased in number in the second case. Diaphragma muscles contained abundant amounts of rods in both cases. The cardiac musculature showed a few rods only in the first patient, who had developed heart insufficiency 11 months prior to death. Immunohistochemical analysis showed that rods did not contain desmin or ubiquitin.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 248 (1987), S. 527-530 
    ISSN: 1432-0878
    Keywords: Epididymal ultrastructure ; Peroxidase ; Protein transport ; Fluid-phase endocytosis ; Sprague-Dawley rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Experiments were performed to clarify the debate over the entry of circulating proteins into the epididymal lumen by use of the marker horseradish peroxidase (HRP). Epididymal tubules from the caput epididymidis of the rat were immersed in medium TC 199 containing HRP (3.5 mg/ ml) for 5 min to 3 h at 33° C. Sections were examined for the presence of tracer within the epithelial cells by electron microscopy. From 5 min to 3 h, vesicles containing peroxidase reaction products were found throughout the cytoplasm of the principal cells. Vesicles occurred close to both the basal and apical membranes, and many were found opening into the interstitial space and lumen, depending on the length of incubation. By 5 min labelled vesicles were infrequently found in the apical part of the cells. Reaction product was observed in the epididymal lumen adhering to the microvilli from 30 min of incubation onwards. At all periods of incubation peroxidase was present at the base of the epithelium and between the cells, but it was never found within the tight junctional complexes, and no reaction deposits were found within epithelial cells of tubules incubated in the absence of peroxidase. It is concluded that large molecules leaving the capillaries may enter the epididymal lumen in the caput by means of fluid-phase endocytosis.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0878
    Keywords: Photoperiod ; Pituitary gland, Pars tuberalis ; TSH ; Immunohistochemistry ; Phodopus sungorus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Certain secretory cells in the hypophysial pars tuberalis of the Djungarian hamster display marked circannual structural alterations. The present investigation deals with the immunohistochemical properties of this cell group. A distinct TSH-like immunoreactivity was found in secretory cells of this type in the pars tuberalis of animals exposed to long photoperiods, whereas under short photoperiods the TSH-like immunoreactivity was nearly absent. In the pars distalis, the number and distribution of TSH-positive cells did not differ significantly between animals maintained under long and under short photoperiods. LH-and FSH-positive cells could not be detected in the pars tuberalis, but they are clearly present in the pars distalis of both groups of hamsters. Our immunocytochemical results suggest that photoperiodic stimuli influence the secretory activity of TSH-like immunoreactive cells in the pars tuberalis. A connection with the neuroendrocrine-thyroid axis is discussed.
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1432-0878
    Keywords: Hypophyseal pars tuberalis ; (TSH), Thyrotropin ; Immunocytochemistry ; Photoperiod ; Phodopus sungorus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Specific secretory cells in the hypophyseal pars tuberalis of Djungarian hamsters maintained under different photoperiods were investigated immunocytochemically by means of the colloidal gold technique using antibodies against rat thyrotropin (TSH). Secretory cells of animals kept under long photoperiods (LD16:8) showed positive staining of secretory granules (diameters 90–130 nm), whereas other intracellular structures were free of immunoreactivity. In animals kept under short photoperiods (LD8:16) secretory cells displayed increased numbers of secretory granules, but these organelles were devoid of immunoreactivity. In contrast, immunoreactivity of thyrotropes in the pars distalis did not differ between the two groups of animals investigated. The present results confirm earlier light-microscopical studies that in the pars tuberalis specific secretory cells show TSH-like immunoreactivity; however, they differ in their reactivity pattern from classical thyrotropes in the pars distalis.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 267 (1992), S. 209-214 
    ISSN: 1432-0878
    Keywords: Testis ; Sulfhydryl oxidase ; Hypospermatogenesis ; Sertoli cell integrity ; Immunocytochemistry ; Man
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary Sulfhydryl oxidase (SOx) immunoreactivity was investigated in the seminiferous epithelium of human biopsy material from the testes of 33 adult men with disturbed fertility. SOx immunoreactivity was expressed in normal seminiferous epithelium in type-A spermatogonia (27±4% of all spermatogonia) (n=4), in spermatocytes and round spermatids. Mature spermatozoa as well as Sertoli cells were unlabelled. within the interstitium, Leydig cells were immunopositive. In biopsies of oligozoospermic men showing hypospermatogenesis (n=24), an increase in labelled spermatogonia up to more than 90% was observed in biopsies, where seminiferous epithelia revealed only spermatogonia and Sertoli cells. Within the group of oligozoospermic patients there was a significant increase of labelled spermatogonia from 43±13% (〉20 mill/ejaculate) (n=7) to 55±16% ( 20 and 〉20 mill/ejaculate) (n=6) to 68±8% (〈5 mill/ejaculate) (n=11) and a significant (P=0.01) decrease of score count from 7.0±2.7 to 2.0±1.8. In this group the increase of labelled spermatogonia was correlated with sperm concentrations in the ajaculate (correlation coefficient: r=-0.6). In biopsies of azoospermic patients showing maturation arrest at the level of spermatocytes or spermatids (n=5) the percentage of labelled spermatogonia was within the range of 24% to 59%. Immunoreactivity in Sertoli cells was only found in single degenerating cells and in tubules showing Sertoli Cell Only Syndrome (SCO) without lumen formation. Sertoli cells within immature seminiferous cords were immunonegative, indicating that Sertoli cell SOx immunoreactivity is rather a sign of physiological alterations in degenerating cells than dependent on the stage of differentiation. Leydig cells did not show changes of immunoreactivity in any biopsy. It is concluded that SOx expression in spermatogonia may serve as a marker for spermatogenic efficiency.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Cell & tissue research 253 (1988), S. 631-637 
    ISSN: 1432-0878
    Keywords: Epididymal ultrastructure ; Transcytosis ; Protein transport ; Fluid-phase endocytosis ; Epididymal arterial perfusion ; Rat (Sprague-Dawley) ; Golden hamster ; Mouse (CB6/F1)
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Summary The transport of protein across the cells of the epididymal epithelium was studied using horseradish peroxidase. Transient vascular perfusion of the epididymis of the rat and golden hamster was achieved by pulsatile retrograde infusion into the testicular artery. Peroxidase was found in the interstitium and in the epithelium, located in vesicles, vacuoles and multivesicular bodies of principal, clear and apical cells. Similar findings were obtained in mice after systemic injection of the tracer. In the rat, discharge to the lumen was confirmed by the appearance of enzyme activity in luminal fluid from the caput epididymidis after local injection. The extent of transport amounted to no more than what has been considered leakage in physiological experiments, and the time-course of appearance complemented that found by electron microscopy. The level of transcytosis after pulsatile administration of peroxidase in vivo, as judged from the occurrence of tracer in the epithelium, was much less than that obtained during constant immersion in vitro. The protein was present in multivesicular bodies of principal cells and in vesicles of clear cells at short times after presentation in vitro, when it could not have arrived by endocytosis from the lumen. This suggests that routing of basal endocytic vesicles to the lysosomal apparatus occurs.
    Type of Medium: Electronic Resource
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