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151.
Wortmann SB Vaz FM Gardeitchik T Vissers LE Renkema GH Schuurs-Hoeijmakers JH Kulik W Lammens M Christin C Kluijtmans LA Rodenburg RJ Nijtmans LG Grünewald A Klein C Gerhold JM Kozicz T van Hasselt PM Harakalova M Kloosterman W Barić I Pronicka E Ucar SK Naess K Singhal KK Krumina Z Gilissen C van Bokhoven H Veltman JA Smeitink JA Lefeber DJ Spelbrink JN Wevers RA Morava E de Brouwer AP 《Nature genetics》2012,44(7):797-802
Using exome sequencing, we identify SERAC1 mutations as the cause of MEGDEL syndrome, a recessive disorder of dystonia and deafness with Leigh-like syndrome, impaired oxidative phosphorylation and 3-methylglutaconic aciduria. We localized SERAC1 at the interface between the mitochondria and the endoplasmic reticulum in the mitochondria-associated membrane fraction that is essential for phospholipid exchange. A phospholipid analysis in patient fibroblasts showed elevated concentrations of phosphatidylglycerol-34:1 (where the species nomenclature denotes the number of carbon atoms in the two acyl chains:number of double bonds in the two acyl groups) and decreased concentrations of phosphatidylglycerol-36:1 species, resulting in an altered cardiolipin subspecies composition. We also detected low concentrations of bis(monoacyl-glycerol)-phosphate, leading to the accumulation of free cholesterol, as shown by abnormal filipin staining. Complementation of patient fibroblasts with wild-type human SERAC1 by lentiviral infection led to a decrease and partial normalization of the mean ratio of phosphatidylglycerol-34:1 to phosphatidylglycerol-36:1. Our data identify SERAC1 as a key player in the phosphatidylglycerol remodeling that is essential for both mitochondrial function and intracellular cholesterol trafficking. 相似文献
152.
153.
J. Mimić-Oka I. Japundžić M. Japundžić 《Cellular and molecular life sciences : CMLS》1972,28(10):1160-1161
Résumé Le pentobarbital provoque un accroissement important de l'activité de la phosphoprotein-phosphatase extramicrosomiale du foie du rat. L'accroissement de l'activité de l'enzyme se produit dejà 20 min après l'administration du médicament (+40%) et dure autant que le traitement (+50–70%). Le niveau de l'induction dépend de la dose du pentobarbital. L'induction est bloquée par l'administration simultanée du cycloheximide ou du SKF 525-A. 相似文献
154.
A. Marušić I. Dikić S. Vukičević M. Marušić 《Cellular and molecular life sciences : CMLS》1992,48(8):783-785
Subcutaneous implantation of demineralized bone matrix (DBM) initiates a sequence of developmental events which culminate in endochondral bone formation. To test the effects of T-cell deficiency on new bone formation, the morphology of DBM-induced bone was examined in rats thymectomized at three weeks of age and in thymectomized or nonthymectomized rats lethally irradiated and reconstituted with syngeneic bone marrow. At 24 days after implantation, bone induction in control rats was appropriate for their age, while thymectomized-irradiated-reconstituted rats and thymectomized rats had significantly more new bone and larger bone marrow space than the controls. In non-thymectomized, irradiated and reconstituted rats, bone induction occurred in only 25% of the animals, compared to 95% in other groups. 相似文献
155.
S. Pajović Z. S. Saičić M. B. Spasić V. M. Petrović J. V. Martinović 《Cellular and molecular life sciences : CMLS》1996,52(3):221-224
The activities of mitochondrial, manganese-containing superoxide dismutase (MnSOD) and cytoplasmic, copper-zinc-containing superoxide dismutase (CuZnSOD) were measured in subcellular fractions of whole brain homogenates prepared from intact and gonadectomized (GDX) male rats, untreated or treated subcutaneously (sc) with a single dose of 2 mg progesterone (P) and/or 5 g estradiol benzoate (EB). Neither MnSOD nor CuZnSOD was affected by the removal of the testes. Similarly, CuZnSOD activity was steady following systemic administration of P and/or EB to intact and GDX animals 2 h or 24 h prior to sacrifice. On the other hand, both P and EB suppressed MnSOD in the brain of either intact or GDX rats. These results suggest involvement of P and EB in the control of MnSOD activity in the brain of male rats. 相似文献
156.
Darinka Koraćević Vidosava Djordjević 《Cellular and molecular life sciences : CMLS》1977,33(8):1010-1011
Summary Trypsin causes an activation of serine sulfhydrase in the liver extracts from intact animals, but inhibits enzyme activity in the liver of ethionine treated rats. Trypsin also decreases an elevation of serine sulfhydrase activity caused by S-adenosylmethionine.This work was supported by the Serbian Medical Research Foundation. 相似文献
157.
M. Marušić 《Cellular and molecular life sciences : CMLS》1979,35(5):683-684
Summary Immunological functions of lethally irradiated mice reconstituted with syngeneic bone marrow cells recover after 5–6 weeks. In mice that had been thymectomized before irradiation and reconstitution, T-cell function is deficient but the B-cell function is preserved.Supported by subcontract No. 3322 from the Biology Division of Oak Ridge National Laboratory of the University of Tennessee, and by the Fulbright-Hays Program. Present address: Department of Physiology, University of Zagreb, Faculty ofOperated by the Union Carbide Corporation for the Department of Energy. 相似文献
158.
Petar V. Grujić 《Foundations of Science》2007,12(1):39-83
We discuss a number of fundamental aspects of modern cosmological concepts, from the phenomenological, observational, theoretical
and epistemic points of view. We argue that the modern cosmology, despite a great advent, in particular in the observational
sector, is yet to solve important problems, posed already by the classical times. In particular the stress is put on discerning
the scientific features of modern cosmological paradigms from the more speculative ones, with the latter immersed in some
aspects deeply into mythological world picture. We finally discuss the principal paradigms, which are present in the modern
cosmological studies and evaluate their epistemic merits. 相似文献
159.
Summary An attempt has been made to systematize the phenomena which are included by the term of Becquerel's effect.Becquerel's photogalvanic effect has been thoroughly investigated on systems composed of organic redox-dyes and organic acceptors, especially regarding the speed of changes in potential and the influence of reducing and oxidizing agents. Possibilities of increasing and stabilizing the electromotor forces of the investigated photogalvanic cells have been ascertained. It has been proved that the well known rules governing inhibition of chemical reaction apply to the investigated systems. 相似文献
160.
Effect of electroshock upon nucleic acid concentrations in various parts of cat brain 总被引:2,自引:0,他引:2
Lj. Mihailović B. D. Janković M. Petković K. Isaković 《Cellular and molecular life sciences : CMLS》1958,14(4):144-145
Zusammenfassung Beim Elektroschock kommt es zu bedeutenden Erniedrigungen der Konzentration von RNA in verschiedenen kortikalen und subkortikalen Teilen des zentralen Nervensystems der Katze, während sich die Konzentration von DNA in normalen Grenzen hält. 相似文献