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  • 1
    ISSN: 1520-4995
    Source: ACS Legacy Archives
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1520-4995
    Source: ACS Legacy Archives
    Topics: Biology , Chemistry and Pharmacology
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1546-1718
    Source: Nature Archives 1869 - 2009
    Topics: Biology , Medicine
    Notes: [Auszug] Collagen VI is an extracellular matrix protein that forms a microfilamentous network in skeletal muscles and other organs. Inherited mutations in genes encoding collagen VI in humans cause two muscle diseases, Bethlem myopathy and Ullrich congenital muscular dystrophy. We previously generated ...
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Journal of bioenergetics and biomembranes 23 (1991), S. 679-688 
    ISSN: 1573-6881
    Keywords: Mitochondria ; permeability transition ; cycloporin A ; phenylarsine oxide
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Physics
    Notes: Abstract This paper reports an investigation on the effects of the hydrophobic, bifunctional SH group reagent phenylarsine oxide (PhAsO) on mitochondrial membrane permeability. We show that PhAsO is a potent inducer of the mitochondrial permeability transition in a process which is sensitive to both the oxygen radical scavanger BHT and to cyclosporin A. The PhAsO-induced permeability transition is stimulated by Ca2+ but takes place also in the presence of EGTA in a process that maintains its sensitivity to BHT and cyclosporin A. Our findings suggest that, at variance from other known inducers of the permeability transition, PhAsO reacts directly with functional SH groups that are inaccessible to hydrophilic reagents in the absence of Ca2+.
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Journal of bioenergetics and biomembranes 28 (1996), S. 131-138 
    ISSN: 1573-6881
    Keywords: Mitochondrial channels ; permeability transition pore ; calcium channels ; cyclosporin A ; ryanodine receptor
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Physics
    Notes: Abstract Mitochondria from a variety of sources possess an inner membrane channel, the permeability transition pore. The pore is a voltage-dependent channel, activated by matrix Ca2+ and inhibited by matrix H+, which can be blocked by cyclosporin A, presumably after binding to mitochondrial cyclophilin. The physiological function of the permeability transition pore remains unknown. Here we evaluate its potential role as a fast Ca2+ release channel involved in mitochondrial and cellular Ca2+ homeostasis. We (i) discuss the theoretical and experimental reasons why mitochondria need a fast, inducible Ca2+ release channel; (ii) analyze the striking analogies between the mitochondrial permeability transition pore and the sarcoplasmic reticulum ryanodine receptor-Ca2+ release channel; (iii) argue that the permeability transition pore can act as a selective release channel for Ca2+ despite its apparent lack of selectivity for the transported speciesin vitro; and (iv) discuss the importance of mitochondria in cellular Ca2+ homeostasis, and how disruption of this function could impinge upon cell viability, particularly under conditions of oxidative stress.
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Journal of bioenergetics and biomembranes 31 (1999), S. 335-345 
    ISSN: 1573-6881
    Keywords: Mitochondrial channels ; respiratory chain ; complex I ; ubiquinone ; cell death ; disease ; aging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Physics
    Notes: Abstract This review summarizes recent progress on the regulation of the mitochondrial permeabilitytransition pore, an inner membrane channel that may play a role in cell death. We brieflycover its key control points as emerged over the last few years from studies on isolatedmitochondria; and describe in some detail our recent results indicating that the pore is modulatedby the respiratory chain complex I and can be specifically blocked by selected ubiquinoneanalogs. We discuss the potential relevance of these findings for the structural definition ofthe permeability transition pore and illustrate the pharmacological perspectives they offer indiseases where mitochondrial dysfunction is suspected to play a key role.
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  • 7
    ISSN: 1573-6881
    Keywords: Mitochondrial permeability transition ; cyclosporin A ; cyclosporin analogs ; transmembrane potential ; membrane surface potential ; lipid mediators
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Physics
    Notes: Abstract The mitochondrial permeability transition pore allows solutes with a m.w. ≲1500 to equilibrate across the inner membrane. A closed pore is favored by cyclosporin A acting at a high-affinity site, which may be the matrix space cylophilin isozyme. Early results obtained with cyclosporin A analogs and metabolites support this hypothesis. Inhibition by cyclosporin does not appear to require inhibition of calcineurin activity; however, it may relate to inhibition of cyclophilin peptide bond isomerase activity. The permeability transition pore is strongly regulated by both the membrane potential (Δψ) and ΔpH components of the mitochondrial protonmotive force. A voltage sensor which is influenced by the disulfide/sulhydryl state of vicinal sulfhydryls is proposed to render pore opening sensitive to Δψ. Early results indicate that this sensor is also responsive to membrane surface potential and/or to surface potential gradients. Histidine residues located on the matrix side of the inner membrane render the pore responsive to ΔpH. The pore is also regulated by several ions and metabolites which act at sites that are interactive. There are many analogies between the systems which regulate the permeability transition pore and the NMDA receptor channel. These suggest structural similarities and that the permeability transition pore belongs to the family of ligand gated ion channels.
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Wireless networks 3 (1997), S. 435-437 
    ISSN: 1572-8196
    Source: Springer Online Journal Archives 1860-2000
    Topics: Electrical Engineering, Measurement and Control Technology , Computer Science
    Notes: Abstract The generation and propagation of radio frequency (RF) electromagnetic waves was first demonstrated by HeinrichHertz in 1888. A few years later, Guglielmo Marconi succeeded in transmitting, wirelessly, a radio signal over a longterrestrial distance in 1896 and then across the Atlantic Ocean in 1901. The number of devices and systems that emitRF radiation has been increasing at an accelerating rate ever since. Some have estimated that there were over 80 millionusers of mobile telephones by the end of 1996. Indeed, wireless communication service is sweeping the world and hasbrought instant, two-way radio communication to many people constantly on the move. The concept of personal communication systems (PCS) aims at providing two-way communication services, speechand data to individual users, indoors or out. Its goal is to establish a mass network for mobile communications andto provide a competitive alternative to the conventional wired public switched telecommunication network. The wide-spreadimpact of this new technology has raised concerns about the safety of human exposure to RF energy emittedby these telecommunication devices. Clearly, we need a better understanding of the biological effects of RF electro-magneticfield so that we can safeguard against possible harm to the general population and enhance its beneficialuses. Within the last few years there has been a resurgence of research effort to achieve better and more quantitativeunderstanding of the relationships between the biological effects of RF radiation and the physical variables that maycause them. Some results are beginning to appear in the literature. This special issue is intended to provide anoverview of the current status of our scientific understanding and to present recent advances coming from variousresearch laboratories.
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Molecular and cellular biochemistry 184 (1998), S. 379-391 
    ISSN: 1573-4919
    Keywords: myocytes ; ischemia-reperfusion ; hypoxia ; cyclosporin ; membrane permeability ; channels ; word ; phrase
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Medicine
    Notes: Abstract Many pathological conditions can be the cause or the consequence of mitochondrial dysfunction. For instance anoxia, which is initiated by a critical reduction of oxygen availability for mitochondrial oxidations, is followed by a wide variety of mitochondrial alterations. A crucial role in the evolution of cell injury is to be attributed to the direction of operation of the F0F1 ATPase, which may turn mitochondria into the major consumers of cellular ATP in the futile attempt to restore the proton electrochemical gradient. On the other hand, functional mitochondria can paradoxically accelerate or exacerbate cell damage. This concept is particularly relevant for the ischemic myocardium. Indeed, inhibition of the respiratory chain or addition of uncouplers of oxidative phosphorylation can both limit the extent of enzyme release in the intact heart and prevent the onset of irreversible morphological changes in isolated myocytes. From studies on different tissues in a variety of pathological conditions a general consensus emerges on the role of intracellular Ca2+ overload as a pivotal link between cellular alterations and mitochondrial dysfunction. Oxidative phosphorylation is reduced by a massive mitochondrial uptake of Ca2+, resulting in a vicious cycle whereby the reduced ATP availability is followed by a failure of the mechanisms which extrude Ca2+ from the sarcoplasm. In addition, the rise in [Ca2+]i could promote opening of the cyclosporin-sensitive mitochondrial permeability transition pore, leading to a sudden ΔΨm dissipation. Here, we review the changes in intracellular and intramitochondrial ionic homeostasis occurring during ischemia and reperfusion. In particular, we evaluate the potential contribution of the permeability transition pore to cellular damage and discuss the mechanisms which can determine the cellular fate from a mitochondrial point of view.
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Journal of bioenergetics and biomembranes 14 (1982), S. 387-403 
    ISSN: 1573-6881
    Keywords: Mitochondrial volume oscillations ; electroneutral H+/TPA+ exchange
    Source: Springer Online Journal Archives 1860-2000
    Topics: Biology , Chemistry and Pharmacology , Physics
    Notes: Abstract Mitochondria incubated aerobically in the presence of tetrapropylammonium and weak acids and in the presence of trace amounts of tetraphenylboron undergo a series of damped oscillations reflecting cycles of osmotic swelling and shrinkage. The matrix volume changes are consequent to transport of tetrapropylammonium catalytically stimulated by tetraphenylboron. The amplitude and frequency of the oscillations increase with the concentration of tetrapropylammonium, as required for critical rates and extents of ion influx. Addition of bovine serum albumin abolishes both the uptake of tetrapropylammonium and the oscillations. Volume oscillations are paralleled by cyclic activation and depression of the respiratory rate. Two lines of evidence suggest that the train of damped oscillations depends on the cyclic activation of an electroneutral exchange of H+ with organic cations rather than on cyclic uncoupling. First, further increase of cation permeability due to a pulse of tetraphenylboron, after initiation of cation efflux, restores cation influx. Second, addition of Mg2+, which abolishes the oscillations, has a much more marked inhibitory effect on the process of cation efflux than on cation influx. Conversely, addition of A23187, which removes membrane-bound Mg2+, promotes cation efflux and thus the oscillations. It is suggested that, in the present system, stretching of the inner membrane and Mg2+ depletion result in activation of an electroneutral H+/organic cation exchange, and that cyclic activation of this reaction results in damped oscillations.
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