Disease relevance of Bile Ducts, Intrahepatic

• While the pathologic mechanisms responsible for organ-specific tissue damage in primary biliary cirrhosis (PBC) remain an enigma, it has been suggested that the pathology is mediated by autoreactive T cells infiltrating the intrahepatic bile ducts [1].
• Studies were carried out in a family in which two children with cholestasis due to intrahepatic bile duct anomalies were shown to have increased amounts of the cholic acid precursor, 3alpha, 7alpha, 12alpha-trihydorxy-5beta-cholestan-26-oic acid (THCA) [2].
• Primary biliary cirrhosis (PBC) is an autoimmune liver disease characterized by destruction of intrahepatic bile ducts [3].
• Mutations of human jagged 1 (JAG1) gene are responsible for Alagille Syndrome (AGS), whose 2 main symptoms are intrahepatic bile duct hypoplasia and pulmonary stenosis [4].
• These cases suggest that cholestatic jaundice in non-cirrhotic PBC may be secondary to extensive "premature" or accelerated intrahepatic bile duct loss [5].

High impact information on Bile Ducts, Intrahepatic

• BACKGROUND & AIMS: We report a novel approach to study biliary water, bile acid, and HCO(3)(-) transport: the microperfusion of intrahepatic bile duct units (IBDUs) isolated from normal rat liver [6].
• METHODS: Small and large cholangiocytes or intrahepatic bile duct units (IBDUs) were isolated from normal rats, and gene expression for the apical Na+-dependent bile acid transporter (ABAT) and the 14-kilodalton ileal cytosolic binding protein (IBABP) was assessed by ribonuclease-protection assays [7].
• RESULTS: The predominant site of CFTR messenger RNA and immunoreactivity in liver is the intrahepatic bile duct [8].
• The data demonstrate for the first time that intrahepatic bile duct epithelial cells participate in receptor-mediated endocytosis and raise the possibility that they are a target for epidermal growth factor [9].
• When rat liver was stained in situ for epidermal growth factor receptor, reaction product was observed by immunoelectron microscopy exclusively on the basal surface of the plasma membrane of the intrahepatic bile duct epithelial cells [9].

Chemical compound and disease context of Bile Ducts, Intrahepatic

• However, five of six infants with paucity of intrahepatic bile ducts, two of six infants with cholestasis secondary to total parenteral nutrition, and one infant with cholangiolitis did not show evidence of gastrointestinal excretion [10].

Biological context of Bile Ducts, Intrahepatic

• The identification of annexin V with predominantly intrahepatic bile ducts, is of significant interest because of the multiple roles of annexin V, including that of membrane cytoskeletal interactions during transport and apoptosis [11].
• We characterized fetal liver derived from 13-21 days of gestation (E13-E21). pi GST was detected in the E18 hepatoblasts, which form the intrahepatic bile ducts, while CK19 was detected at E19 [12].
• Storage of alpha-1-antitrypsin in intrahepatic bile duct cells in alpha-1-antitrypsin deficiency (Pi Z phenotype) [13].
• Two weeks' exposure to PCP at a concentration of 600 ppm in the diet increased 8-oxodeoxyguanosine (8-oxodG) levels in liver nuclear DNA, and cell proliferation quantified by bromodeoxyuridine (BrdU) incorporation in epithelial cells of intrahepatic bile ducts as well as hepatocytes [14].
• Furthermore, TGF-alpha might play a role in morphogenesis and regeneration of intrahepatic bile ducts [15].

Anatomical context of Bile Ducts, Intrahepatic

• Generation of monoclonal antibodies to murine bile duct epithelial cells: identification of annexin V as a new marker of small intrahepatic bile ducts [11].
• One of three intrahepatic bile duct cancers, one of two common bile duct cancers, and one of two ampullary cancers had mutations in the p16Ink4/CDKN2 gene [16].
• Immunohistochemistry revealed low levels of TGF-beta 1 in normal intrahepatic bile duct epithelium [17].
• In conclusion, NTPDase2 is expressed in a novel cellular compartment surrounding intrahepatic bile ducts, namely portal fibroblasts [18].
• NTPDase2 was expressed in the periportal region surrounding intrahepatic bile ducts, whereas NTPDase1 was found in hepatic arteries, portal veins, and hepatic central veins, consistent with its known vascular distribution [18].

Associations of Bile Ducts, Intrahepatic with chemical compounds

• We measured the effect of insulin on (1) secretin-stimulated cAMP levels in cholangiocytes and duct expansion in intrahepatic bile duct units (IBDUs) in the absence or presence of BAPTA/AM, H7 or rottlerin and (2) bile flow [19].
• CONCLUSIONS: Cooling of the bile ducts with a cold 5% glucose isotonic solution significantly protects the intrahepatic bile ducts from damages caused by the heat generated by RF when performed close to the bile ducts [20].
• In hepatolithiasis, bilirubin-calcium stones are packed in multiple irregularly dilated intrahepatic bile ducts [21].
• Taurocholate prevents the loss of intrahepatic bile ducts due to vagotomy in bile duct-ligated rats [22].
• The calibre of the intrahepatic bile ducts was correctly reported in 66 patients (98 per cent) [23].

Gene context of Bile Ducts, Intrahepatic

• In hepatolithiasis, MUC1, MUC2, and MUC5 apomucins were also focally expressed in 33%, 64%, and 89%, respectively, of the large intrahepatic bile ducts examined, whereas such expression was infrequent in normal livers and in extrahepatic biliary obstruction [24].
• Due to exon skipping, Pkhd1ex40 mice express a modified Pkhd1 transcript and develop severe malformations of intrahepatic bile ducts [25].
• In this study, we therefore investigated in situ expression of matrix metalloproteinases (MMP) and tissue inhibitors of MMP (TIMP) during human intrahepatic bile duct development, using 32 human fetal livers [26].
• In 10/22 (45 per cent) patients with PBC and 3/7 (43 per cent) patients with PSC, B7-2, but not B7-1, was expressed on the epithelial cells of small intrahepatic bile ducts and bile ductules [27].
• Altered mucin gene expression in stone-containing intrahepatic bile ducts and cholangiocarcinomas [28].

Analytical, diagnostic and therapeutic context of Bile Ducts, Intrahepatic

• By in situ hybridization, FasL mRNA were frequently and strongly expressed in biliary dysplasia compared with non-neoplastic intrahepatic bile duct [29].
• Delineation of the IRHV is significant in hepatectomy, detection of tumor thrombus in hepatocellular carcinoma, Budd-Chiari syndrome, and dilatation of the intrahepatic bile ducts [30].
• METHOD: Cystic and intrahepatic bile duct (IHBD) abnormalities identified by MR cholangiography in 93 ADPKD patients were studied retrospectively [31].
• Prior to a diagnostic needle biopsy a PTCD was performed in this case presenting with dilated intrahepatic bile ducts and having a history of Billroth II operation [32].
• Because primary biliary cirrhosis (PBC) is characterized by targeting of small intrahepatic bile ducts by autoreactive T cells, plasma concentrations of sCD40L were investigated using enzyme-linked immunosorbent assay (ELISA) to measure plasma concentrations of sCD40L in 12 patients with PBC and 12 healthy controls [33].

References