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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 202 (1972), S. 21-36 
    ISSN: 1432-1459
    Keywords: Hydrocephalus, Experimental ; Ethylnitrosourea ; Experimental Hydrocephalus ; Rat
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Zusammenfassung 25 mg/kg Äthylnitrosoharnstoff wurden am 9 1/2. Tag der Schwangerschaft SD-JCL-Ratten injiziert. Die Feten wurden am Termin excidiert und mit dem Stereomikroskop untersucht. 78% der Feten zeigten einen Hydrocephalus. Die histologische Untersuchung in Stufenschnitten des Gehirns zeigte eine Stenose des Aquädukts ohne subependymale Gliose, ödematöse Plexus chorioidei mit prallgefüllten Gefäßen, spärlichem Bindegewebe und geschwollenen Epithelzellen, Verdünnung der telencephalen Wandungen mit schwach entwickelter granulärer Schicht und in der ependymalen Zone verbleibenden Neuroblasten. Die Entwicklung der jungen Ratten wurde studiert. 75% der neugeborenen Ratten zeigten klinisch gewisse Hinweise auf den Hydrocephalus. Stark betroffene Tiere wurden zunehmend somnolent und/oder gelähmt und starben meist vor Ende der 4. Woche. Der Eiweißgehalt des Liquors war nennenswert vermehrt. Es wurden subdurale, subarachnoidale und intraventrikuläre Blutungen beobachtet und als wahrscheinliche Todesursache angesehen. Die histologische Untersuchung der hydrocephalen Jungtiere ergab eine Abflachung der Ependymzellschicht und pralle Füllung corticaler Gefäße von der 2. Woche an. In späteren Stadien wurde eine Dickenabnahme der periventrikulären weißen Substanz, im besonderen im Bereiche des Occipitalhornes nachgewiesen. Im verdünnten Pallium waren Vacuolenbildungen des perineuralen Gewebes zu sehen, Verbreiterung der Virchow-Robinschen Räume und eine dem Status spongiosus ähnliche Degeneration. Verschiedene Formen ependymaler Wucherung wurden gesehen. Die vorliegende Studie stellt die erste postnatale Beobachtung von transplacentar verursachtem kongenitalem Hydrocephalus in der Ratte dar. Die hier beschriebene Technik erwies sich als einfaches und reproduzierbares Mittel, um einen kongenitalen Hydrocephalus in der Ratte zu erzeugen.
    Notes: Summary SD-JCL rats were injected with 25 mg/kg of ethylnitrosourea on day 9 1/2 of gestation. Fetuses were excised near term and examined under dissecting microscope. 78% of viable fetuses were found to be hydrocephalic. On the histologic study of the serial sections of the brain, a stenosis of the Sylvian aqueduct without subependymal gliosis, edematous choroid plexus with engorged vessels, scanty connective tissue and swollen epithelium, thinning of the telencephalic wall with poorly developed granular layers and the neuroblasts remaining in the ependymal zone, were the characteristic findings in the hydrocephalic fetuses. The natural course of the treated young rats was studied. 75% of the newborn rats showed some clinical evidence of hydrocephalus. Severely affected rats became progressively lethargic and/or paralytic, and died usually before the end of 4th week. Protein content of the cerebrospinal fluid was significantly increased. Subdural, subarachnoid and intraventricular hemorrhages were observed and suspected to be the cause of death. On the histological examination of hydrocephalic youngs, flattening of the ependymal cells and engorgement of the cortical vessels were observed as early as in 2-week-old youngs. In more advanced stages, there was noted the thinning of the periventricular white matter, especially around the occipital horn. The thin pallium showed vacuolization of the perineural tissue, enlargement of the Virchow-Robin space and the status spongiosus-like degeneration. Various types of ependymal proliferation was seen. The present study represents the first postnatal observation of the transplacentally induced congenital hydrocephalus in the rat. The present technique proved to be a simple and reproducible model of producing a congenital hydrocephalus in the rat.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-1203
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Medicine
    Notes: Abstract OPLL (ossification of the posterior longitudinal ligament of the spine) is a common form of human myelopathy with a prevalence of as much as 4% in a variety of ethnic groups. To clarify the genetic factors that predispose to OPLL, we have studied ttw (tiptoe walking), a mouse model that presents ectopic ossification of the spinal ligaments similar to OPLL and have found that the ttw phenotype is caused by the nonsense mutation of the gene encoding nucleotide pyrophosphatase (NPPS), a membrane-bound glycoprotein thought to produce inorganic pyrophosphate, a major inhibitor of calcification and mineralization. To investigate a possible role of NPPS in the etiology of OPLL, we have examined its genetic variations in OPLL patients. A total of 323 OPLL patients was screened by means of polymerase chain reaction/single-strand conformation polymorphism analysis covering all the exons and their surrounding introns, plus about 1.5-kb of the promoter region. We identified ten nucleotide variations in the NPPS gene; five of the alterations caused amino-acid substitutions, and two of them were found specifically in OPLL patients. Subsequently, we performed an association study using these variations and found a significant association of an allele, viz., a deletion of T at a position 11 nucleotides upstream from the splice acceptor site of intron 20 (IVS20–11delT), with OPLL; the proportion of the individuals having this deletion was significantly higher (P = 0.0029) in OPLL patients than in controls, indicating that those who have this variation may be more susceptible to the abnormal ossification of the spinal ligaments. Thus, our study suggests that NPPS plays an important role in the etiology of human OPLL.
    Type of Medium: Electronic Resource
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