254 results found
    1. Stem Cells and Regenerative Medicine
    2. Immunology and Inflammation

    MHC-compatible bone marrow stromal/stem cells trigger fibrosis by activating host T cells in a scleroderma mouse model

    Yoko Ogawa et al.
    PDGFRα+ Sca-1+ bone marrow stromal/stem cells in whole bone marrow grafts can trigger the onset of autoimmune-related fibrosis in a mouse model of scleroderma.
    1. Chromosomes and Gene Expression

    Therapeutic effects of telomerase in mice with pulmonary fibrosis induced by damage to the lungs and short telomeres

    Juan Manuel Povedano et al.
    Telomerase gene therapy represents a novel effective treatment for pulmonary fibrosis associated with short telomeres by improving pulmonary function, decreasing inflammation and accelerating fiber disappearance in fibrotic lungs.
    1. Human Biology and Medicine

    Nanoscale dysregulation of collagen structure-function disrupts mechano-homeostasis and mediates pulmonary fibrosis

    Mark G Jones et al.
    Nanoscale changes to individual collagen fibrils drive lung fibrosis.
    1. Stem Cells and Regenerative Medicine

    Cell non-autonomous functions of S100a4 drive fibrotic tendon healing

    Jessica E Ackerman et al.
    Inhibition of S100a4 represents a novel anti-fibrotic target to improve tendon healing.
    1. Structural Biology and Molecular Biophysics

    Cystic fibrosis drug ivacaftor stimulates CFTR channels at picomolar concentrations

    László Csanády, Beáta Töröcsik
    Aqueous solubility of cystic fibrosis drug ivacaftor is ~200-fold lower, whereas the potency of its stimulatory effect on the CFTR channel is >100-fold higher, than reported, and is fully reversible.
    1. Human Biology and Medicine
    2. Immunology and Inflammation

    ENaC-mediated sodium influx exacerbates NLRP3-dependent inflammation in cystic fibrosis

    Thomas Scambler et al.
    Dysfunction and overexpression of ENaC-mediated sodium influx exacerbates activation of NLRP3-inflammasome mediated inflammation in cells with CF-associated mutations and is modulated by inhibition of these amiloride-sensitive sodium (Na+) channels.
    1. Human Biology and Medicine

    Identification of functionally distinct fibro-inflammatory and adipogenic stromal subpopulations in visceral adipose tissue of adult mice

    Chelsea Hepler et al.
    Fibro-inflammatory progenitors represent a subpopulation of perivascular cells in visceral adipose tissues of mice that promote inflammation and fibrosis.
    1. Computational and Systems Biology
    2. Human Biology and Medicine

    Unravelling druggable signalling networks that control F508del-CFTR proteostasis

    Ramanath Narayana Hegde et al.
    Analysis of the mechanism of action of cystic fibrosis corrector drugs reveals signalling pathways potently controlling the proteostasis of the main disease-relevant CFTR mutant.
    1. Developmental Biology

    A structure-based extracellular matrix expansion mechanism of fibrous tissue growth

    Nicholas S Kalson et al.
    Tendon fibroblast cells lay down a collagen matrix template during embryogenesis, and the expansion of this matrix drives post-natal tissue growth.
    1. Cell Biology
    2. Developmental Biology

    Matrix metalloproteinase 14 is required for fibrous tissue expansion

    Susan H Taylor et al.
    The membrane type I-matrix metalloproteinase is essential for the release of collagen fibrils from plasma membrane fibripositors, which is required for the transition from embryonic to postnatal tendon development.

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