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  • 1
    Electronic Resource
    Electronic Resource
    [s.l.] : Nature Publishing Group
    Nature 184 (1959), S. 889-890 
    ISSN: 1476-4687
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Notes: [Auszug] The diurnal variations of auroral frequency for the two stations are very similar in form and characterized by primary morning and secondary evening maxima. Such bi-modal frequency curves have been derived before and have been the subject of comment by Hulbert2, who, however, failed to ascribe any ...
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1573-7446
    Keywords: ATP synthase ; cattle ; ceroid-lipofuscinosis ; lysosomes ; proteolipid ; subunit c
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The ceroid-lipofuscinoses (Batten's disease) are a group of recessively inherited lysosomal storage diseases of children and animals in which there is intracellular accumulation of a fluorescent lipopigment in a wide variety of cells. Lipopigment bodies isolated from pancreas, liver, kidney and brain tissue from a heifer affected with ceroid-lipofuscinosis contained between 55 and 62% protein. A dominant component comigrated on LDS-PAGE with the major low molecular weight protein stored in ovine ceroid-lipofuscinosis. It was identified by amino acid sequence and mass spectroscopy as the full subunit c of mitochondrial ATP synthase, normally found only in the inner mitochondrial membrane, where it is estimated to account for 2–4% of the membrane protein. In pancreatic lipopigment it accounted for at least 40% of the total lipopigment mass and this storage was considered specific to the disease. No other mitochondrial proteins were found in storage bodies. These results are similar to those found in studies on the ovine and the late infantile and juvenile human forms of the disease. It is concluded that bovine ceroid-lipofuscinosis is also a proteolipid proteinosis in which subunit c of mitochondrial ATP synthase is specifically stored in lysosome derived organelles.
    Type of Medium: Electronic Resource
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