Hepatic abnormalities in Long-Evans Cinnamon (LEC) rats, an animal model of Wilson disease (WD), were restored by the expression of the human ATP7B cDNA under the control of CAG promoter. Expression of ATP7B transcript and protein in the liver of the transgenic rats resulted in the restoration of biosynthesis of ...

With the epidemic proportions of obesity worldwide and the concurrent prevalence of hepatic steatosis, there is an urgent need for better understanding the intrinsic mechanism of hepatic steatosis, especially the changes of gene expression underlying the development of hepatic steatosis and its associated ...

... such an abnormal polysaccharide appears to be related to radiation-induced alterations in the relative activities of glycogen synthesizing enzymes ...

The Havanese is a toy breed that presents with a wide range of developmental abnormalities. Skeletal defects, particularly osteochondrodysplasia (OCD), are the most frequently observed anomalies. Cataracts, liver shunts, heart murmurs, and missing incisors are also common in this breed. Estimates of heritability and complex segregation analyses were carried out to evaluate ...

Background/AimsWilson disease (WD) is a disorder of copper transport caused by mutations within the ATP7B gene. WD is phenotypically variable and can present with predominantly hepatic or neurologic manifestations. The mechanisms responsible for this variability are unknown. GP73, a Golgi membrane protein, is expressed in hepatocytes in response to acute ...

Chronic hepatitis B virus (HBV) infection has been strongly associated with hepatocellular carcinoma . HBV encodes an oncogenic hepatitis B virus X protein (HBx), which is a multifunctional regulator that modulates signal transduction, transcription, cell cycle progress, protein degradation, apoptosis, and genetic stability through direct and indirect ...

withdrawal Meningitis Metabolic abnormalities such as low blood glucose Sedative overdose Subdural hematoma (bleeding under the skull) Wernicke-Korsakoff syndrome Hepatic...

Patients with diabetes suffer disproportionately from impaired lipid metabolism and cardiovascular disease, but the relevant roles of insulin resistance and hyperglycemia in these processes are unclear. Transcription factor FoxO1 is regulated dually by insulin and nutrients. In this study, we addressed the hypothesis that, in addition to its established role to regulate ...

Patients with diabetes suffer disproportionately from impaired lipid metabolism and cardiovascular disease, but the relevant roles of insulin resistance and hyperglycemia in these processes are unclear. Transcription factor FoxO1 is regulated dually by insulin and nutrients. In this study, we addressed the hypothesis that, in addition to its established role to regulate ...

Liver transplantation has become a standard option in the management of patients with end-stage liver disease. It is now evident that the most common etiology of long-term graft dysfunction is the recurrence of the primary liver disease. Autoimmune liver disorders such as autoimmune hepatitis, primary biliary cirrhosis, and primary sclerosing cholangitis recur between 15 to ...

Loss of ABCA1 activity in Tangier disease (TD) is associated with abnormal apoB lipoprotein (Lp) metabolism in addition to the complete absence of high density lipoprotein (HDL). We used hepatocyte-specific ABCA1 knock-out (HSKO) mice to test the hypothesis that hepatic ABCA1 plays dual roles in regulating Lp metabolism and nascent HDL formation. HSKO mice ...

Loss of ABCA1 activity in Tangier disease (TD) is associated with abnormal apoB lipoprotein (Lp) metabolism in addition to the complete absence of high density lipoprotein (HDL). We used hepatocyte-specific ABCA1 knock-out (HSKO) mice to test the hypothesis that hepatic ABCA1 plays dual roles in regulating Lp metabolism and nascent HDL formation. HSKO mice ...

Decreased amounts of the serum copper-binding protein caeruloplasmin (hypocaeruloplasminaemia) are one of the characteristic abnormalities of copper metabolism that are found in all neonatal mammals. In the present study we have investigated the mechanism responsible for hypocaeruloplasminaemia found in neonatal guinea pigs. Northern-blot analysis of guinea-pig liver RNA ...

Mice deficient in acyl-CoA:diacylglycerol acyltransferase 1 (DGAT1), a key enzyme in triacylglycerol (TG) biosynthesis, are resistant to high-fat (HF) diet-induced hepatic steatosis and obesity. DGAT1-deficient (Dgat1^?/?) mice have no defect in quantitative absorption of dietary fat; however, they have abnormally high levels of TG ...

... (Author). Descriptors : *HEPATITIS VIRUSES, *TISSUE CULTURE CELLS, PHAGOCYTES, CELLS(BIOLOGY), PATHOLOGY, VIRUSES, GROWTH ...

... adverse events � Increases in hepatic transaminases and bilirubin � Metabolic disorders such as hyperglycemia and hyperlipidemia, and abnormal fat ...

... Accession Number : ADA273490. Title : Expression and Diagnostic Utility of Hepatitis E Virus Putative Structural Proteins Expressed in Insect Cells. ...

BackgroundMutations in the Wnt signalling pathway molecule ?-catenin are associated with liver cancer.AimsOur aim was to confirm the effects of stabilized ?-catenin on liver growth, identify whether those effects were reversible and cell autonomous or non-cell autonomous and to model ?-catenin-induced liver cancer in mice.MethodsUsing a liver-specific inducible promoter, we generated transgenic ...

The longest open reading frame of PKHD1 (polycystic kidney and hepatic disease 1), the autosomal recessive polycystic kidney disease (ARPKD) gene, encodes a single-pass, integral membrane protein named polyductin or fibrocystin. A fusion protein comprising its intracellular C-terminus, FP2, was previously used to raise a polyclonal antiserum shown to detect polyductin in ...

This symposium includes articles on fluid and electrolyte disorders in cirrhosis; hepatic encephalopathy syndrome; coagulation abnormalities in liver disease; fulminant hepatic failure; variceal hemorrhage; and congenital hepatic fibrosis with sepsis and ...

Tracer quantities of colloidal $sup 198$Au were used to estimate the hepatic blood flow in normal children and in children with active or progressive chronic hepatitis and also to obtain scintigrams of the liver. In active chronic hepatitis a significant decrease in HBF values was observed, suggesting that the method may be ...

The peroxisome proliferator-activated receptor ? (PPAR?) coactivator 1? (PGC-1?) is a highly inducible transcriptional coactivator implicated in the coordinate regulation of genes encoding enzymes involved in hepatic fatty acid oxidation, oxidative phosphorylation, and gluconeogenesis. The present study sought to assess the effects of chronic PGC-1? deficiency on metabolic ...

PDZK1 is a four PDZ domain-containing scaffold protein that binds to scavenger receptor class B, type I (SR-BI), the high density lipoprotein receptor, by its first PDZ domain (PDZ1). PDZK1 knock-out mice exhibit a >95% decrease in hepatic SR-BI protein and consequently an ?70% increase in plasma cholesterol in abnormally large high density ...

Scavenger receptor SR-BI significantly contributes to HDL cholesterol metabolism and atherogenesis in mice. However, the role of SR-BI may not be as pronounced in humans due to cholesteryl ester transfer protein (CETP) activity. To address the impact of CETP expression on the adverse effects associated with SR-BI deficiency, we cross-bred our SR-BI conditional knock-out mouse ...

1 NK cells in chronic hepatitis C and hepatitis B Fine Characterization of Intra-hepatic NK cells Expressing Natural Killer Receptors in Chronic Hepatitis B and C Paula Bonorino1,2* , Muhammad Ramzan 1. The proportions of intra-hepatic NK cells ...

Two necropsy cases of Down syndrome are reported. These showed transient abnormal myelopoiesis accompanying characteristic hepatic sinusoidal fibrosis. Numerous megakaryocytes were found in the liver of one case, but not in the other. Only eight cases of Down syndrome with simultaneous occurrence of hepatic fibrosis and transient ...

Total hepatic venous drainage into the left atrium is an extremely uncommon abnormality. We present a patient in whom the hepatic veins drained into the left atrium in the absence of other intracardiac or extracardiac anomalies. Surgical correction of the anomalous hepatic venous connection was performed by ...

Idiopathic neonatal hepatitis is the uncommon syndrome of prolonged obstructive jaundice associated with giant cell transformation in the liver and patent bile ducts. Either hepatitis virus or an inherited abnormality has been suggested as a likely pathogenic agent for the syndrome. Recent observation of discordance for idiopathic ...

Hepatic stellate cells (HSCs) are important part of the local 'stem cell niche' for hepatic progenitor cells (HPCs) and hepatocytes. However, it is unclear as to whether the products of activated HSCs are required to attenuate hepatocyte injury, enhance liver regeneration, or both. In this study, we performed 'loss of function' studies by depleting ...

Type 1 diabetes mellitus is caused by severe insulin deficiency secondary to the autoimmune destruction of pancreatic ? cells. Patients need to be controlled by periodic insulin injections to prevent the development of ketoacidosis, which can be fatal. Sustained, low-level expression of the rat insulin 1 gene from the liver of severely diabetic rats was achieved by in vivo ...

AMPK (AMP-activated protein kinase) has been suggested to be a central player regulating FA (fatty acid) metabolism through its ability to regulate ACC (acetyl-CoA carboxylase) activity. Nevertheless, its involvement in insulin resistance- and TD2 (Type 2 diabetes)-associated dyslipidaemia remains enigmatic. In the present study, we employed the Psammomys obesus gerbil, a well-established model of ...

Hepatic structure and function of 22 unselected patients with early syphilis was assessed. In 20 (91%) routine hepatic tests or bromsulphalein retention showed mild non-specific abnormalities. Minor changes in hepatic structure were present in 12 (55%), in three of whom intrahepatic spirochaetes were found. The ...

Hepatic artery infusion chemotherapy is used in the treatment of certain selected hepatic tumors, especially metastatic adenocarcinoma of the colon. Chemical cholecystitis has been recognized recently as a complication of hepatic artery infusion chemotherapy. The authors performed hepatobiliary scans on ten patients receiving ...

There is evidence that both cellular and humoral components of the immune response are required for viral clearance to occur in chronic hepatitis B. Recent studies demonstrated that CD30 molecule, a member of the tumour necrosis factor superfamily of membrane cytokine receptors, is expressed on, and released as a soluble molecule (sCD30) by activated T ...

The objectives of this work are to further our knowledge of the pathogenesis of hepatitis A virus (HAV) infection in man, and to develop recombinant expression vectors for hepatitis A virus antigens that can be used to stimulate mucosal immunity. Two vira...

An investigation on human peripheral blood lymphocyte chromosomes in chronic active hepatitis was carried out. A higher percentage of chromatid and chromosome lesions was recorded in all patients studied as compared with control groups�normal individuals, healthy subjects who had suffered from acute viral hepatitis, patients with alcoholic liver disease, ...

Background/AimsThe importance of epigenetic changes in etiology and pathogenesis of disease has been increasingly recognized. However, the role of epigenetic alterations in the genesis of hepatic steatosis and cause of individual susceptibilities to this pathological state are largely unknown.MethodsMale inbred C57BL/6J and DBA/2J mice were fed a lipogenic methyl-deficient ...

We aimed to characterize the primary abnormalities associated with fat accumulation and vulnerability to hepatocellular injury of obesity-related fatty liver. We performed functional analyses and comparative transcriptomics of isolated primary hepatocytes from livers of obese insulin-resistant Zucker rats (comprising mild to severe hepatic steatosis) and ...

Folate deficiency is present in most patients with alcoholic liver disease (ALD), whereas folate regulates and alcoholism perturbs intrahepatic methionine metabolism, and S-adenosyl-methionine prevents the development of experimental ALD. Our studies explored the hypothesis that abnormal methionine metabolism is exacerbated by folate deficiency and promotes the development of ...

Forkhead box O1 (FoxO1) is a transcription factor that mediates the inhibitory effect of insulin on target genes in hepatic metabolism. Hepatic FoxO1 activity is up-regulated to promote glucose production during fasting and is suppressed to limit postprandial glucose excursion after meals. Increased FoxO1 activity augments the ...

Forkhead box O1 (FoxO1) is a transcription factor that mediates the inhibitory effect of insulin on target genes in hepatic metabolism. Hepatic FoxO1 activity is up-regulated to promote glucose production during fasting and is suppressed to limit postprandial glucose excursion after meals. Increased FoxO1 activity augments the ...

Chronic hepatitis C (CHC) is generally a slowly progressive disease, but some factors associated with rapid progression have been identified. Steatosis, independently of its metabolic or viral origin, leads to liver injury and fibrosis. There are suggestions that hepatitis C virus may contribute to a wide spectrum of metabolic disturbances-namely, ...

The gene encoding the transcriptional coactivator peroxisome proliferator-activated receptor-? coactivator-1? (PGC-1?) was targeted in mice. PGC-1? null (PGC-1?^?/?) mice were viable. However, extensive phenotyping revealed multi-system abnormalities indicative of an abnormal energy metabolic phenotype. The postnatal growth of heart ...

High-density lipoproteins (HDLs) are major carriers of cholesterol in the bloodstream and are critical in regulating cholesterol homeostasis in vivo. The first molecularly well-defined and physiologically relevant HDL receptor to be characterized was the scavenger receptor class B type I (SR-BI), a cell surface glycoprotein most highly expressed in liver and steroidogenic ...

Although toxocaral granulomatous hepatitis (TGH) characterized with a dominant-Th2 type immune response is a self-limiting disease, little is known concerning the role of fibrosis-related cytokine transforming growth factor-beta 1 (TGF-beta 1) in pathogenesis of TGH. A detailed histological and quantitatively immunohistochemical analysis of TGF-beta 1, alpha-smooth muscle ...