Browsing by Subject "Liver"
Now showing items 1-20 of 24
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A population model of folate-mediated one-carbon metabolism.
(Nutrients, 2013-07-05)BACKGROUND: Previous mathematical models for hepatic and tissue one-carbon metabolism have been combined and extended to include a blood plasma compartment. We use this model to study how the concentrations of metabolites ... -
A whole-cell and single-channel study of the voltage-dependent outward potassium current in avian hepatocytes.
(J Gen Physiol, 1988-02)Voltage-dependent membrane currents were studied in dissociated hepatocytes from chick, using the patch-clamp technique. All cells had voltage-dependent outward K+ currents; in 10% of the cells, a fast, transient, ... -
Alglucosidase alfa treatment alleviates liver disease in a mouse model of glycogen storage disease type IV.
(Mol Genet Metab Rep, 2016-12)Patients with progressive hepatic form of GSD IV often die of liver failure in early childhood. We tested the feasibility of using recombinant human acid-α glucosidase (rhGAA) for treating GSD IV. Weekly intravenously injection ... -
Characterization of a canine model of glycogen storage disease type IIIa.
(Dis Model Mech, 2012-11)Glycogen storage disease type IIIa (GSD IIIa) is an autosomal recessive disease caused by deficiency of glycogen debranching enzyme (GDE) in liver and muscle. The disorder is clinically heterogeneous and progressive, and ... -
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 ... -
Gene therapy for glycogen storage diseases.
(Human molecular genetics, 2019-10)The focus of this review is the development of gene therapy for glycogen storage diseases (GSDs). GSD results from the deficiency of specific enzymes involved in the storage and retrieval of glucose in the body. Broadly, ... -
Hepatic histological findings in suspected drug-induced liver injury: systematic evaluation and clinical associations.
(Hepatology (Baltimore, Md.), 2014-02)Drug-induced liver injury (DILI) is considered to be a diagnosis of exclusion. Liver biopsy may contribute to diagnostic accuracy, but the histological features of DILI and their relationship to biochemical parameters and ... -
Hepatic mitochondrial dysfunction is a feature of Glycogen Storage Disease Type Ia (GSDIa).
(Scientific reports, 2017-03)Glycogen storage disease type Ia (GSDIa, von Gierke disease) is the most common glycogen storage disorder. It is caused by the deficiency of glucose-6-phosphatase, an enzyme which catalyses the final step of gluconeogenesis ... -
Immunodominant liver-specific expression suppresses transgene-directed immune responses in murine pompe disease.
(Hum Gene Ther, 2012-05)Pompe disease can be treated effectively, if immune tolerance to enzyme replacement therapy (ERT) with acid α-glucosidase (GAA) is present. An adeno-associated viral (AAV) vector carrying a liver-specific regulatory cassette ... -
Impact of gene variants on sex-specific regulation of human Scavenger receptor class B type 1 (SR-BI) expression in liver and association with lipid levels in a population-based study.
(BMC Med Genet, 2010-01-19)BACKGROUND: Several studies have noted that genetic variants of SCARB1, a lipoprotein receptor involved in reverse cholesterol transport, are associated with serum lipid levels in a sex-dependent fashion. However, the mechanism ... -
Increased Glutaminolysis Marks Active Scarring in Nonalcoholic Steatohepatitis Progression.
(Cellular and molecular gastroenterology and hepatology, 2020-01)<h4>Background & aims</h4>Nonalcoholic steatohepatitis (NASH) occurs in the context of aberrant metabolism. Glutaminolysis is required for metabolic reprograming of hepatic stellate cells (HSCs) and liver fibrogenesis in ... -
Low-dose imaging of liver diseases through neutron stimulated emission computed tomography: Simulations in GEANT4
(2013)Neutron stimulated emission computed tomography (NSECT) is a non-invasive, tomographic imaging technique with the ability to locate and quantify elemental concentration in a tissue sample. Previous studies have shown that ... -
Magnetic resonance microscopy.
(Anal Cell Pathol (Amst), 2012) -
Natural Progression of Canine Glycogen Storage Disease Type IIIa.
(Comp Med, 2016-02)Glycogen storage disease type IIIa (GSD IIIa) is caused by a deficiency of glycogen debranching enzyme activity. Hepatomegaly, muscle degeneration, and hypoglycemia occur in human patients at an early age. Long-term complications ... -
Neutron Stimulated Emission Computed Tomography: Optimization of Acquisition Parameters Using Resolution and Dosimetry in the Context of Liver and Breast Cancers
(2013)Proposed is a method for investigating optimal acquisition parameters in NSECT, neutron stimulated emission computed tomography, for good image quality and low dose for diagnosing liver and breast cancers. These parameters ... -
Parental dietary seleno-L-methionine exposure and resultant offspring developmental toxicity.
(Aquatic toxicology (Amsterdam, Netherlands), 2016-01)Selenium (Se) leaches into water from agricultural soils and from storage sites for coal fly ash. Se toxicity causes population and community level effects in fishes and birds. We used the laboratory aquarium model fish, ... -
Pathogenesis of growth failure and partial reversal with gene therapy in murine and canine Glycogen Storage Disease type Ia.
(Molecular Genetics and Metabolism, 2013-06)Glycogen Storage Disease type Ia (GSD-Ia) in humans frequently causes delayed bone maturation, decrease in final adult height, and decreased growth velocity. This study evaluates the pathogenesis of growth failure and the ... -
Quantifiable biomarkers of normal aging in the Japanese medaka fish (Oryzias latipes).
(PLoS One, 2010-10-11)BACKGROUND: Small laboratory fish share many anatomical and histological characteristics with other vertebrates, yet can be maintained in large numbers at low cost for lifetime studies. Here we characterize biomarkers associated ... -
Spectral diffusion: an algorithm for robust material decomposition of spectral CT data.
(Phys Med Biol, 2014-11-07)Clinical successes with dual energy CT, aggressive development of energy discriminating x-ray detectors, and novel, target-specific, nanoparticle contrast agents promise to establish spectral CT as a powerful functional ... -
The influence of hepatic function on prostate cancer outcomes after radical prostatectomy.
(Prostate Cancer Prostatic Dis, 2010-06)Prostate growth is dependent on circulating androgens, which can be influenced by hepatic function. Liver disease has been suggested to influence prostate cancer (CaP) incidence. However, the effect of hepatic function on ...
