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  • 1
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    Abnormal Ras signaling in Costello syndrome (CS) negatively regulates enamel formation (2014)
    Oxford University Press
    Details
    Publication Date: 2014-01-11
    Description: RASopathies are syndromes caused by gain-of-function mutations in the Ras signaling pathway. One of these conditions, Costello syndrome (CS), is typically caused by an activating de novo germline mutation in HRAS and is characterized by a wide range of cardiac, musculoskeletal, dermatological and developmental abnormalities. We report that a majority of individuals with CS have hypo-mineralization of enamel, the outer covering of teeth, and that similar defects are present in a CS mouse model. Comprehensive analysis of the mouse model revealed that ameloblasts, the cells that generate enamel, lacked polarity, and the ameloblast progenitor cells were hyperproliferative. Ras signals through two main effector cascades, the mitogen-activated protein kinase (MAPK) and phosphatidylinositol-3-kinase (PI3K) pathways. To determine through which pathway Ras affects enamel formation, inhibitors targeting either PI3K or MEK 1 and 2 (MEK 1/2), kinases in the MAPK pathway, were utilized. MEK1/2 inhibition rescued the hypo-mineralized enamel, normalized the ameloblast polarity defect and restored normal progenitor cell proliferation. In contrast, PI3K inhibition only corrected the progenitor cell proliferation phenotype. We demonstrate for the first time the central role of Ras signaling in enamel formation in CS individuals and present the mouse incisor as a model system to dissect the roles of the Ras effector pathways in vivo .
    Print ISSN: 0964-6906
    Electronic ISSN: 1460-2083
    Topics: Biology , Medicine
    Published by Oxford University Press
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  • 2
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    Nucleotide sequences of the human and mouse atrial natriuretic factor genes (1984)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 1984-12-07
    Description: Mouse and human atrial natriuretic factor (ANF) genes have been cloned and their nucleotide sequences determined. Each ANF gene consists of three coding blocks separated by two intervening sequences. The 5' flanking sequences and those encoding proANF are highly conserved between the two species, while the intervening sequences and 3' untranslated regions are not. The conserved sequences 5' of the gene may play an important role in the regulation of ANF gene expression.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Seidman, C E -- Bloch, K D -- Klein, K A -- Smith, J A -- Seidman, J G -- AI-18436/AI/NIAID NIH HHS/ -- HL-070208/HL/NHLBI NIH HHS/ -- New York, N.Y. -- Science. 1984 Dec 7;226(4679):1206-9.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/6542248" target="_blank"〉PubMed〈/a〉
    Keywords: Animals ; *Atrial Natriuretic Factor ; Base Sequence ; Cloning, Molecular ; Gene Expression Regulation ; Genes ; Heart Atria/metabolism ; Humans ; Mice ; Natriuretic Agents ; Protein Precursors/genetics ; Proteins/*genetics ; Receptors, Glucocorticoid/metabolism
    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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    A novel mechanism of somatic rearrangement predicted by a human T-cell antigen receptor beta-chain complementary DNA (1985)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 1985-06-07
    Description: The T-cell antigen receptor is a cell surface molecule vital in mediating the cellular immune response. The arrangement and rearrangement of the gene segments encoding the beta-chain polypeptide of the receptor are similar to those of immunoglobulin gene segments. The two constant region genes of the human T-cell antigen receptor are 8 kilobases apart with a cluster of joining segments located 5' of each constant region gene. Although most beta-chain gene rearrangements involve the variable, diversity, and joining segments, analysis of a beta-chain complementary DNA clone suggests the occasional occurrence of another type of rearrangement.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Duby, A D -- Klein, K A -- Murre, C -- Seidman, J G -- AI-19438/AI/NIAID NIH HHS/ -- New York, N.Y. -- Science. 1985 Jun 7;228(4704):1204-6.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/3839095" target="_blank"〉PubMed〈/a〉
    Keywords: Base Sequence ; DNA/genetics ; Genes ; Humans ; Macromolecular Substances ; Receptors, Antigen, T-Cell/*genetics ; Recombination, Genetic
    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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  • 4
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    Specific surface: determination and relevance (2002)
    Canadian Science Publishing
    Details
    Publication Date: 2002-02-01
    Description: Specific surface captures the combined effects of particle size and slenderness in a measurement that is independent and complementary to grain-size distribution. There are various methods to measure specific surface, including gas adsorption in dry conditions and selective molecular absorption in aqueous suspensions. The measurement procedure can have an important effect on measured values, yet such sensitivity is informative in itself. The amount of surface in a soil mass determines the balance between surface-related forces and gravimetricskeletal forces acting on a soil particle, affects fabric formation, supports rich energy coupling mechanisms, governs conduction, and controls sorption and retardation during chemical diffusion.Key words: specific surface, surface area, methylene blue, gas adsorption, fabric, Atterberg limits, grain-size distribution.
    Print ISSN: 0008-3674
    Electronic ISSN: 1208-6010
    Topics: Geosciences
    Published by Canadian Science Publishing
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