15 Result(s)
within
Ronald G. Crystal
Science, Humanities and Social Sciences, multidisciplinary
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Article
Open AccessExpression and processing of mature human frataxin after gene therapy in mice
Friedreich’s ataxia is a degenerative and progressive multisystem disorder caused by mutations in the highly conserved frataxin (FXN) gene that results in FXN protein deficiency and mitochondrial dysfunction. ...
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Article
Open AccessImpaired differentiation of small airway basal stem/progenitor cells in people living with HIV
With highly active anti-retroviral therapy (HAART), higher incidence of airway abnormalities is common in the HIV population consistent with the concept of accelerated lung “aging”. Our previous findings demon...
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Article
Open AccessExtracellular vesicles from human airway basal cells respond to cigarette smoke extract and affect vascular endothelial cells
The human airway epithelium lining the bronchial tree contains basal cells that proliferate, differentiate, and communicate with other components of their microenvironment. One method that cells use for interc...
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Article
Open AccessHIV induces airway basal progenitor cells to adopt an inflammatory phenotype
Despite the introduction of anti-retroviral therapy, chronic HIV infection is associated with an increased incidence of other comorbidities such as COPD. Based on the knowledge that binding of HIV to human air...
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Open AccessIncreased airway iron parameters and risk for exacerbation in COPD: an analysis from SPIROMICS
Levels of iron and iron-related proteins including ferritin are higher in the lung tissue and lavage fluid of individuals with chronic obstructive pulmonary disease (COPD), when compared to healthy controls. W...
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Open AccessIntermittent exposure to whole cigarette smoke alters the differentiation of primary small airway epithelial cells in the air-liquid interface culture
Cigarette smoke (CS) is the leading risk factor to develop COPD. Therefore, the pathologic effects of whole CS on the differentiation of primary small airway epithelial cells (SAEC) were investigated, using ce...
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Article
Open AccessUntargeted Metabolite Profiling of Cerebrospinal Fluid Uncovers Biomarkers for Severity of Late Infantile Neuronal Ceroid Lipofuscinosis (CLN2, Batten Disease)
Late infantile neuronal ceroid lipofuscinosis (CLN2 disease) is a rare lysosomal storage disorder caused by a monogenetic deficiency of tripeptidyl peptidase-1 (TPP1). Despite knowledge that lipofuscin is the hal...
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Open AccessWhole-exome sequencing identifies common and rare variant metabolic QTLs in a Middle Eastern population
Metabolomics-genome-wide association studies (mGWAS) have uncovered many metabolic quantitative trait loci (mQTLs) influencing human metabolic individuality, though predominantly in European cohorts. By combin...
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Open AccessA call for transparent reporting to optimize the predictive value of preclinical research
Deficiencies in methods reporting in animal experimentation lead to difficulties in reproducing experiments; the authors propose a set of reporting standards to improve scientific communication and study design.
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Article
Altered chloride ion channel kinetics associated with the ΔF508 cystic fibrosis mutation
CYSTIC fibrosis is associated with a defect in epithelial chloride ion transport (reviewed in refs 1, 2) which is caused by mutations in a membrane protein called CFTR (cystic fibrosis transmembrane conductanc...
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Article
Activated human monocytes express the c-sis proto-oncogene and release a mediator showing PDGF-like activity
Current ideas about the mechanism of wound healing and the pathogenesis of atherosclerosis, pulmonary fibrosis and hepatic fibrosis suggest a central role for the mononuclear phagocyte in attracting and/or sti...
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Article
Synthesis in E. coli of α1-antitrypsin variants of therapeutic potential for emphysema and thrombosis
The primary function of α1-antitrypsin (α1-AT), an antiprotease produced by the liver, is the inhibition of neutrophil elastase, a protease capable of hydrolysing most connective tissue components1–4. The importa...
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Article
Fibroblasts degrade newly synthesised collagen within the cell before secretion
COLLAGEN, the major extracellular protein of most tissues, is considered a relatively inert macromolecule, with a turnover rate slower than that of most proteins1. There is, however, a portion of the total collag...
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Article
Rigid control of synthesis of collagen Types I and III by cells in culture
THE constituents of the extracellular matrix have a fundamental role in the structure and function of all tissues. A major insight into the composition of this matrix came with the discovery1–3 that interstitial ...
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Activation of alveolar macrophage collagenase by a neutral protease secreted by the same cell
MAMMALIAN collagenase is a proteolytic enzyme which specifically cleaves native triple helical collagen at physiological pH and temperature. As with other extracellular proteases, there is increasing evidence tha...
