Browsing Harvard Medical School by Title
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Defective lymphoid organogenesis underlies the immune deficiency caused by a heterozygous S32I mutation in IκBα
(The Rockefeller University Press, 2015)Patients with ectodermal dysplasia with immunodeficiency (ED-ID) caused by mutations in the inhibitor of NF-κB α (IκBα) are susceptible to severe recurrent infections, despite normal T and B cell numbers and intact in vitro ... -
Defective NKT Cell Activation by CD1d+ TRAMP Prostate Tumor Cells Is Corrected by Interleukin-12 with alpha-Galactosylceramide
(Public Library of Science, 2010)Numerical and functional defects of invariant natural killer T cells (iNKT) have been documented in human and mouse cancers, resulting in a defect in IFN production in several malignancies. iNKT cells recognize glycolipids ... -
Defective sister chromatid cohesion is synthetically lethal with impaired APC/C function
(Nature Pub. Group, 2015)Warsaw breakage syndrome (WABS) is caused by defective DDX11, a DNA helicase that is essential for chromatid cohesion. Here, a paired genome-wide siRNA screen in patient-derived cell lines reveals that WABS cells do not ... -
Defective sphingosine-1-phosphate receptor 1 (S1P1) phosphorylation exacerbates TH17-mediated autoimmune neuroinflammation
(2014)Sphingosine-1-phosphate (S1P) signaling regulates lymphocyte egress from lymphoid organs into systemic circulation. Sphingosine phosphate receptor 1 (S1P1) agonist, FTY-720 (Gilenya™) arrests immune trafficking and prevents ... -
Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1α/LRP130 complex
(Cold Spring Harbor Laboratory Press, 2006)Leigh syndrome French Canadian variant (LSFC) is an autosomal recessive neurodegenerative disorder due to mutation in the LRP130 (leucine-rich protein 130 kDa) gene. Unlike classic Leigh syndrome, the French Canadian variant ... -
Defects in muscle branched-chain amino acid oxidation contribute to impaired lipid metabolism
(Elsevier, 2016)Objective: Plasma levels of branched-chain amino acids (BCAA) are consistently elevated in obesity and type 2 diabetes (T2D) and can also prospectively predict T2D. However, the role of BCAA in the pathogenesis of insulin ... -
A Defensin from the Model Beetle Tribolium castaneum Acts Synergistically with Telavancin and Daptomycin against Multidrug Resistant Staphylococcus aureus
(Public Library of Science, 2015)The red flour beetle Tribolium castaneum is a common insect pest and has been established as a model beetle to study insect development and immunity. This study demonstrates that defensin 1 from T. castaneum displays in ... -
Deficiencies of GM-CSF and Interferon γ Link Inflammation and Cancer
(Rockefeller University Press, 2003)Chronic inflammation contributes to carcinogenesis, but the underlying mechanisms are poorly understood. We report that aged granulocyte-macrophage colony stimulating factor (GM-CSF)-deficient mice develop a systemic lupus ... -
Deficiency of cathepsin S reduces atherosclerosis in LDL receptor–deficient mice
(American Society for Clinical Investigation, 2003)Human atherosclerotic lesions overexpress the lysosomal cysteine protease cathepsin S (Cat S), one of the most potent mammalian elastases known. In contrast, atheromata have low levels of the endogenous Cat S inhibitor ... -
Deficiency of FcεR1 increases body weight gain but improves glucose tolerance in diet-induced obese mice
(Endocrine Society, 2015)Prior studies demonstrated increased plasma immunoglobulin E (IgE) in diabetic patients, but the direct participation of IgE in diabetes or obesity remains unknown. This study found that plasma IgE levels correlated inversely ... -
Deficiency of Lipid Phosphatase SHIP Enables Long-Term Reconstitution of Hematopoietic Inductive Bone Marrow Microenvironment
(Elsevier BV, 2013)A dysfunctional bone marrow (BM) microenvironment is thought to contribute to the development of hematologic diseases. However, functional replacement of pathologic BM microenvironment through BM transplantation has not ... -
Deficiency of Phosphoinositide 3-Kinase Enhancer Protects Mice From Diet-Induced Obesity and Insulin Resistance
(American Diabetes Association, 2010)OBJECTIVE: Phosphoinositide 3-kinase enhancer A (PIKE-A) is a proto-oncogene that promotes tumor growth and transformation by enhancing Akt activity. However, the physiological functions of PIKE-A in peripheral tissues are ... -
Deficiency of Retinaldehyde Dehydrogenase 1 Induces BMP2 and Increases Bone Mass In Vivo
(Public Library of Science, 2013)The effects of retinoids, the structural derivatives of vitamin A (retinol), on post-natal peak bone density acquisition and skeletal remodeling are complex and compartment specific. Emerging data indicates that retinoids, ... -
Deficient biological motion perception in schizophrenia: results from a motion noise paradigm
(Frontiers Media S.A., 2013)Background:: Schizophrenia patients exhibit deficient processing of perceptual and cognitive information. However, it is not well-understood how basic perceptual deficits contribute to higher level cognitive problems in ... -
Deficits in High- (>60 Hz) Gamma-Band Oscillations During Visual Processing in Schizophrenia
(Frontiers Media S.A., 2013)Current theories of the pathophysiology of schizophrenia have focused on abnormal temporal coordination of neural activity. Oscillations in the gamma-band range (>25 Hz) are of particular interest as they establish ... -
A defined synthetic substrate for serum-free culture of human stem cell derived cardiomyocytes with improved functional maturity identified using combinatorial materials microarrays
(2016)Cardiomyocytes from human stem cells have applications in regenerative medicine and can provide models for heart disease and toxicity screening. Soluble components of the culture system such as growth factors within serum ... -
Defining a Radiomic Response Phenotype: A Pilot Study using targeted therapy in NSCLC
(Nature Publishing Group, 2016)Medical imaging plays a fundamental role in oncology and drug development, by providing a non-invasive method to visualize tumor phenotype. Radiomics can quantify this phenotype comprehensively by applying image-characterization ... -
Defining an Electrophysiological Phenotype and Increasing Survival in Amyotrophic Lateral Sclerosis Patient-Derived Motor Neurons
(2015-06-08)Amyotrophic Lateral Sclerosis (ALS) is a rapidly progressive and almost universally fatal neurodegenerative disease of the human motor nervous system1. Multiple lines of evidence from ALS patients and animal models have ... -
Defining an International Standard Set of Outcome Measures for Patients With Hip or Knee Osteoarthritis: Consensus of the International Consortium for Health Outcomes Measurement Hip and Knee Osteoarthritis Working Group
(John Wiley and Sons Inc., 2016)Objective: To define a minimum Standard Set of outcome measures and case‐mix factors for monitoring, comparing, and improving health care for patients with clinically diagnosed hip or knee osteoarthritis (OA), with a focus ... -
Defining Clonal Color in Fluorescent Multi-Clonal Tracking
(Nature Publishing Group, 2016)Clonal heterogeneity and selection underpin many biological processes including development and tumor progression. Combinatorial fluorescent protein expression in germline cells has proven its utility for tracking the ...