Browsing by Subject "Mice, Knockout"
Now showing items 1-20 of 88
-
A membrane-associated progesterone-binding protein, 25-Dx, is regulated by progesterone in brain regions involved in female reproductive behaviors.
(Proc Natl Acad Sci U S A, 2000-11-07)The ventromedial hypothalamus (VMH) plays a central role in the regulation of the female reproductive behavior lordosis, a behavior dependent upon the sequential activation of receptors for the ovarian steroid hormones estradiol ... -
Adjunctive β2-agonist treatment reduces glycogen independently of receptor-mediated acid α-glucosidase uptake in the limb muscles of mice with Pompe disease.
(FASEB J, 2014-05)Enzyme or gene replacement therapy with acid α-glucosidase (GAA) has achieved only partial efficacy in Pompe disease. We evaluated the effect of adjunctive clenbuterol treatment on cation-independent mannose-6-phosphate ... -
Adjunctive β2-agonists reverse neuromuscular involvement in murine Pompe disease.
(FASEB J, 2013-01)Pompe disease has resisted enzyme replacement therapy with acid α-glucosidase (GAA), which has been attributed to inefficient cation-independent mannose-6-phosphate receptor (CI-MPR) mediated uptake. We evaluated β2-agonist ... -
Age-related changes in the cellular composition and epithelial organization of the mouse trachea.
(PloS one, 2014-01)We report here senescent changes in the structure and organization of the mucociliary pseudostratified epithelium of the mouse trachea and main stem bronchi. We confirm previous reports of the gradual appearance of age-related, ... -
Altered trabecular bone structure and delayed cartilage degeneration in the knees of collagen VI null mice.
(PLoS One, 2012)Mutation or loss of collagen VI has been linked to a variety of musculoskeletal abnormalities, particularly muscular dystrophies, tissue ossification and/or fibrosis, and hip osteoarthritis. However, the role of collagen ... -
An aberrant SREBP-dependent lipogenic program promotes metastatic prostate cancer.
(Nature genetics, 2018-02)Lipids, either endogenously synthesized or exogenous, have been linked to human cancer. Here we found that PML is frequently co-deleted with PTEN in metastatic human prostate cancer (CaP). We demonstrated that conditional ... -
An immunoglobulin C kappa-reactive single chain antibody fusion protein induces tolerance through receptor editing in a normal polyclonal immune system.
(J Exp Med, 2005-03-07)Understanding immune tolerance mechanisms is a major goal of immunology research, but mechanistic studies have generally required the use of mouse models carrying untargeted or targeted antigen receptor transgenes, which ... -
Angiopoietin receptor TEK mutations underlie primary congenital glaucoma with variable expressivity.
(The Journal of clinical investigation, 2016-07)Primary congenital glaucoma (PCG) is a devastating eye disease and an important cause of childhood blindness worldwide. In PCG, defects in the anterior chamber aqueous humor outflow structures of the eye result in elevated ... -
Antibody formation and mannose-6-phosphate receptor expression impact the efficacy of muscle-specific transgene expression in murine Pompe disease.
(J Gene Med, 2010-11)BACKGROUND: Lysosomal storage disorders such as Pompe disease can be more effectively treated, if immune tolerance to enzyme or gene replacement therapy can be achieved. Alternatively, immune responses against acid α-glucosidase ... -
Autophagy enhances NFκB activity in specific tissue macrophages by sequestering A20 to boost antifungal immunity.
(Nat Commun, 2015-01-22)Immune responses must be well restrained in a steady state to avoid excessive inflammation. However, such restraints are quickly removed to exert antimicrobial responses. Here we report a role of autophagy in an early host ... -
Axonal regrowth after spinal cord injury via chondroitinase and the tissue plasminogen activator (tPA)/plasmin system.
(The Journal of neuroscience : the official journal of the Society for Neuroscience, 2011-10)Spinal cord injury (SCI) causes permanent debilitation due to the inability of axons to grow through established scars. Both the sugar chains and core proteins of chondroitin sulfate proteoglycans (CSPGs) are inhibitory ... -
BCL2 inhibits cell adhesion, spreading, and motility by enhancing actin polymerization.
(Cell Res, 2010-04)BCL2 is best known as a multifunctional anti-apoptotic protein. However, little is known about its role in cell-adhesive and motility events. Here, we show that BCL2 may play a role in the regulation of cell adhesion, spreading, ... -
beta-Arrestin 1 and 2 differentially regulate heptahelical receptor signaling and trafficking.
(Proc Natl Acad Sci U S A, 2001-02-13)The two widely coexpressed isoforms of beta-arrestin (termed beta arrestin 1 and 2) are highly similar in amino acid sequence. The beta-arrestins bind phosphorylated heptahelical receptors to desensitize and target them ... -
beta-Arrestin1 mediates nicotinic acid-induced flushing, but not its antilipolytic effect, in mice.
(J Clin Invest, 2009-05)Nicotinic acid is one of the most effective agents for both lowering triglycerides and raising HDL. However, the side effect of cutaneous flushing severely limits patient compliance. As nicotinic acid stimulates the GPCR ... -
Beta-arrestins regulate atherosclerosis and neointimal hyperplasia by controlling smooth muscle cell proliferation and migration.
(Circ Res, 2008-07-03)Atherosclerosis and arterial injury-induced neointimal hyperplasia involve medial smooth muscle cell (SMC) proliferation and migration into the arterial intima. Because many 7-transmembrane and growth factor receptors promote ... -
Cathelicidin-related antimicrobial peptide mediates skeletal muscle degeneration caused by injury and Duchenne muscular dystrophy in mice.
(Journal of cachexia, sarcopenia and muscle, 2022-12)<h4>Background</h4>Cathelicidin, an antimicrobial peptide, plays a key role in regulating bacterial killing and innate immunity; however, its role in skeletal muscle function is unknown. We investigated the potential role ... -
Characterization of liver GSD IX γ2 pathophysiology in a novel Phkg2-/- mouse model.
(Molecular genetics and metabolism, 2021-07)<h4>Introduction</h4>Liver Glycogen Storage Disease IX is a rare metabolic disorder of glycogen metabolism caused by deficiency of the phosphorylase kinase enzyme (PhK). Variants in the PHKG2 gene, encoding the liver-specific ... -
Complement C4 inhibits systemic autoimmunity through a mechanism independent of complement receptors CR1 and CR2.
(J Exp Med, 2000-11-06)The complement system enhances antibody responses to T-dependent antigens, but paradoxically, deficiencies in C1 and C4 are strongly linked to autoantibody production in humans. In mice, disruption of the C1qa gene also ... -
Defective lymphocyte chemotaxis in beta-arrestin2- and GRK6-deficient mice.
(Proc Natl Acad Sci U S A, 2002-05-28)Lymphocyte chemotaxis is a complex process by which cells move within tissues and across barriers such as vascular endothelium and is usually stimulated by chemokines such as stromal cell-derived factor-1 (CXCL12) acting ... -
Defects of prostate development and reproductive system in the estrogen receptor-alpha null male mice.
(Endocrinology, 2009-01)The estrogen receptor-alpha knockout (ERalphaKO, ERalpha-/-) mice were generated via the Cre-loxP system by mating floxed ERalpha mice with beta-actin (ACTB)-Cre mice. The impact of ERalpha gene deletion in the male reproductive ...
