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  • 1
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    Cell therapy of alpha-sarcoglycan null dystrophic mice through intra-arterial delivery of mesoangioblasts (2003)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 2003-07-12
    Description: Preclinical or clinical trials for muscular dystrophies have met with modest success, mainly because of inefficient delivery of viral vectors or donor cells to dystrophic muscles. We report here that intra-arterial delivery of wild-type mesoangioblasts, a class of vessel-associated stem cells, corrects morphologically and functionally the dystrophic phenotype of virtually all downstream muscles in adult immunocompetent alpha-sarcoglycan (alpha-SG) null mice, a model organism for limb-girdle muscular dystrophy. When mesoangioblasts isolated from juvenile dystrophic mice and transduced with a lentiviral vector expressing alpha-SG were injected into the femoral artery of dystrophic mice, they reconstituted skeletal muscle in a manner similar to that seen in wild-type cells. The success of this protocol was mainly due to widespread distribution of donor stem cells through the capillary network, a distinct advantage of this strategy over previous approaches.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Sampaolesi, Maurilio -- Torrente, Yvan -- Innocenzi, Anna -- Tonlorenzi, Rossana -- D'Antona, Giuseppe -- Pellegrino, M Antonietta -- Barresi, Rita -- Bresolin, Nereo -- De Angelis, M Gabriella Cusella -- Campbell, Kevin P -- Bottinelli, Roberto -- Cossu, Giulio -- 1322/Telethon/Italy -- 463/BI/Telethon/Italy -- New York, N.Y. -- Science. 2003 Jul 25;301(5632):487-92. Epub 2003 Jul 10.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Stem Cell Research Institute, H. S. Raffaele, Via Olgettina 58, 20132 Milan, Italy.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/12855815" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Blood Vessels/cytology/embryology ; Cell Differentiation ; Cell Line ; Cell Movement ; Cytoskeletal Proteins/*genetics/*metabolism ; Dystrophin/metabolism ; Endothelium, Vascular/physiology ; Female ; Femoral Artery ; Genetic Vectors ; Lentivirus/genetics ; Locomotion ; Male ; Membrane Glycoproteins/*genetics/*metabolism ; Mesoderm/cytology ; Mice ; Mice, Knockout ; Mice, Transgenic ; Muscle Contraction ; Muscle Fibers, Skeletal/cytology/physiology ; Muscle, Skeletal/cytology/metabolism/pathology/*physiology ; Muscular Dystrophy, Animal/metabolism/pathology/*therapy ; Regeneration ; Sarcoglycans ; *Stem Cell Transplantation ; Stem Cells/*physiology ; Transfection
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
    Published by American Association for the Advancement of Science (AAAS)
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  • 2
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    Abnormal coronary function in mice deficient in alpha1H T-type Ca2+ channels (2003)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 2003-11-25
    Description: Calcium ion (Ca2+) influx through voltage-gated Ca2+ channels is important for the regulation of vascular tone. Activation of L-type Ca2+ channels initiates muscle contraction; however, the role of T-type Ca2+ channels (T-channels) is not clear. We show that mice deficient in the alpha1H T-type Ca2+ channel (alpha(1)3.2-null) have constitutively constricted coronary arterioles and focal myocardial fibrosis. Coronary arteries isolated from alpha(1)3.2-null arteries showed normal contractile responses, but reduced relaxation in response to acetylcholine and nitroprusside. Furthermore, acute blockade of T-channels with Ni2+ prevented relaxation of wild-type coronary arteries. Thus, Ca2+ influx through alpha1H T-type Ca2+ channels is essential for normal relaxation of coronary arteries.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Chen, Chien-Chang -- Lamping, Kathryn G -- Nuno, Daniel W -- Barresi, Rita -- Prouty, Sally J -- Lavoie, Julie L -- Cribbs, Leanne L -- England, Sarah K -- Sigmund, Curt D -- Weiss, Robert M -- Williamson, Roger A -- Hill, Joseph A -- Campbell, Kevin P -- New York, N.Y. -- Science. 2003 Nov 21;302(5649):1416-8.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Howard Hughes Medical Institute, University of Iowa, Iowa City, IA 52242, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/14631046" target="_blank"〉PubMed〈/a〉
    Keywords: Acetylcholine/pharmacology ; Animals ; Arteries/drug effects/*physiology ; Calcium/*metabolism ; Calcium Channels, T-Type/genetics/*physiology ; Coronary Vessels/drug effects/pathology/*physiology ; Echocardiography ; Electrocardiography ; Endothelium, Vascular/drug effects/physiology ; Female ; Fibrosis ; Ganglia, Spinal/cytology ; Gene Targeting ; Heart/physiology ; Heart Rate ; Male ; Mice ; Mice, Inbred C57BL ; Mice, Knockout ; Muscle, Smooth, Vascular/physiology ; Myocardium/pathology ; Neurons/metabolism ; Nickel/pharmacology ; Nitric Oxide/physiology ; Nitric Oxide Donors/pharmacology ; Nitroprusside/pharmacology ; Patch-Clamp Techniques ; Vasoconstriction/drug effects ; *Vasodilation/drug effects
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
    Published by American Association for the Advancement of Science (AAAS)
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  • 3
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    LARGE glycans on dystroglycan function as a tunable matrix scaffold to prevent dystrophy (2013)
    Nature Publishing Group (NPG)
    Details
    Publication Date: 2013-10-18
    Description: The dense glycan coat that surrounds every cell is essential for cellular development and physiological function, and it is becoming appreciated that its composition is highly dynamic. Post-translational addition of the polysaccharide repeating unit [-3-xylose-alpha1,3-glucuronic acid-beta1-]n by like-acetylglucosaminyltransferase (LARGE) is required for the glycoprotein dystroglycan to function as a receptor for proteins in the extracellular matrix. Reductions in the amount of [-3-xylose-alpha1,3-glucuronic acid-beta1-]n (hereafter referred to as LARGE-glycan) on dystroglycan result in heterogeneous forms of muscular dystrophy. However, neither patient nor mouse studies has revealed a clear correlation between glycosylation status and phenotype. This disparity can be attributed to our lack of knowledge of the cellular function of the LARGE-glycan repeat. Here we show that coordinated upregulation of Large and dystroglycan in differentiating mouse muscle facilitates rapid extension of LARGE-glycan repeat chains. Using synthesized LARGE-glycan repeats we show a direct correlation between LARGE-glycan extension and its binding capacity for extracellular matrix ligands. Blocking Large upregulation during muscle regeneration results in the synthesis of dystroglycan with minimal LARGE-glycan repeats in association with a less compact basement membrane, immature neuromuscular junctions and dysfunctional muscle predisposed to dystrophy. This was consistent with the finding that patients with increased clinical severity of disease have fewer LARGE-glycan repeats. Our results reveal that the LARGE-glycan of dystroglycan serves as a tunable extracellular matrix protein scaffold, the extension of which is required for normal skeletal muscle function.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3891507/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3891507/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Goddeeris, Matthew M -- Wu, Biming -- Venzke, David -- Yoshida-Moriguchi, Takako -- Saito, Fumiaki -- Matsumura, Kiichiro -- Moore, Steven A -- Campbell, Kevin P -- 1RC2NS069521-01/NS/NINDS NIH HHS/ -- 1U54NS053672/NS/NINDS NIH HHS/ -- F32 AR057289-01/AR/NIAMS NIH HHS/ -- T32-DK07690-16/DK/NIDDK NIH HHS/ -- U54 NS053672/NS/NINDS NIH HHS/ -- Howard Hughes Medical Institute/ -- England -- Nature. 2013 Nov 7;503(7474):136-40. doi: 10.1038/nature12605. Epub 2013 Oct 16.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉1] Howard Hughes Medical Institute, Roy J. and Lucille A. Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA [2] Department of Molecular Physiology and Biophysics, Roy J. and Lucille A. Carver College of Medicine, The University of Iowa, Iowa City, Iowa 52242, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/24132234" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; Basement Membrane/metabolism/pathology ; Cell Differentiation ; Cell Line ; Dystroglycans/*chemistry/*metabolism ; Extracellular Matrix/chemistry/*metabolism ; Female ; Humans ; Ligands ; Male ; Mice ; Mice, Inbred C57BL ; Molecular Weight ; Muscle Development ; Muscles/metabolism/pathology ; Muscular Dystrophies/metabolism/pathology/*prevention & control ; Myoblasts ; N-Acetylglucosaminyltransferases/deficiency/genetics/*metabolism ; Neuromuscular Junction/metabolism/pathology ; Phenotype ; Polysaccharides/chemistry/*metabolism
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Published by Nature Publishing Group (NPG)
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  • 4
    Electronic Resource
    Electronic Resource
    Scopolamine permation through human skin in vitro (1976)
    Hoboken, NJ : Wiley-Blackwell
    AIChE Journal 22 (1976), S. 828-832 
    Details
    ISSN: 0001-1541
    Keywords: Chemistry ; Chemical Engineering
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Process Engineering, Biotechnology, Nutrition Technology
    Notes: The sorption and rate of permeation of scopolamine base in human skin have been measured as a function of drug concentration in aqueous solution contacting the stratum corneum surface of the skin. The sorption isotherm is nonlinear, and the apparent penetrant diffusivity computed from steady state permeation data is greater than that estimated from unsteady state (time lag) measurements.By assuming that sorption occurs by both ordinary dissolution and binding of penetrant to immobile sites in the membrane, the experimental sorption isotherm can be predicted, and the disparity between steady state and time lag diffusivities can be reconciled.
    Additional Material: 6 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/aic.690220503
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  • 5
    Electronic Resource
    Electronic Resource
    Effect of dimethyl sulfoxide on drug permeation through human skin (1977)
    Hoboken, NJ : Wiley-Blackwell
    AIChE Journal 23 (1977), S. 810-816 
    Details
    ISSN: 0001-1541
    Keywords: Chemistry ; Chemical Engineering
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Process Engineering, Biotechnology, Nutrition Technology
    Notes: The effect of the presence of dimethyl sulfoxide (in admixture with water) on the sorption and permeation rate of scopolamine base in human skin in vitro has been measured as a function of drug concentration in aqueous solution. The equilibrium sorption of scopolamine by skin from solution appears to be unaffected by the presence of even high concentrations of dimethyl sulfoxide in the solution phase. In the absence of a transdermal gradient of DMSO (or water), the permeability of skin to scopolamine in the presence of DMSO is about twofold higher than in its absence, suggesting that the diffusivity of scopolamine in the stratum corneum is somewhat elevated by the solvating action of DMSO.When, however, a gradient of DMSO concentration is impressed across the skin (irrespective of whether that gradient is of the same or opposite sign to that of the drug), the permeability of the skin to scopolamine is increased by one to two orders of magnitude. Microscopic examination of the skin subjected to such treatment reveals marked swelling, distortion, and intercellular delamination of the stratum corneum, which is only partially reversible following complete extraction with water. These effects are believed due to development of very high osmotic stresses produced within the stratum corneum, as both water and DMSO are transported into the tissue.
    Additional Material: 6 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/aic.690230606
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  • 6
    Electronic Resource
    Electronic Resource
    Application of the ‘dual sorption’ model to drug transport through skin (1980)
    Stamford, Conn. [u.a.] : Wiley-Blackwell
    Polymer Engineering and Science 20 (1980), S. 36-39 
    Details
    ISSN: 0032-3888
    Keywords: Chemistry ; Chemical Engineering
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Chemistry and Pharmacology , Mechanical Engineering, Materials Science, Production Engineering, Mining and Metallurgy, Traffic Engineering, Precision Mechanics , Physics
    Notes: The dual sorption theory has been extended to the transport of drug molecules through human skin in vitro. By assuming that sorption of drug molecules occurs by both dissolution and binding of drug to immobile sites in the skin, the experimental sorption isotherm can be predicted, and the disparity between steady state and time lag diffusivities can be reconciled. Furthermore, the dual sorption model has been used to develop techniques for controlling these sorption transport processes in order to rapidly achieve predictable transdermal drug delivery in vivo.
    Additional Material: 6 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/pen.760200107
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  • 7
    Electronic Resource
    Electronic Resource
    Isolation of predominantly submicron-sized UHMWPE wear particles from periprosthetic tissues (1995)
    Hoboken, NJ : Wiley-Blackwell
    Journal of Biomedical Materials Research 29 (1995), S. 127-131 
    Details
    ISSN: 0021-9304
    Keywords: Chemistry ; Polymer and Materials Science
    Source: Wiley InterScience Backfile Collection 1832-2000
    Topics: Medicine , Technology
    Notes: A method of tissue digestion using sodium hydroxide was applied to the isolation and recovery of ultra-high-molecular-weight polyethylene (UHMWPE) particles from tissues around failed total hip replacements. Density gradient ultracentrifugation of the digested tissues was performed to separate the UHMWPE from cell debris and other particulates. Fourier transform infrared spectroscopy and differential scanning calorimetry (DSC) verified that the recovered particles were UHMWPE. When viewed by scaning electron microscopy, individual particles were clearly observed and were either rounded or elongated. The majority were submicron in size. The application of this method to the study or particles from periprosthetic tissues may elucidate aspects of biomaterial particles size and shape that are important to the biologic response to, and clinical outcome of, total joint replacement. © 1995 John wiley & Sons, Inc.
    Additional Material: 3 Ill.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1002/jbm.820290118
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