Reduced hepatic expression of farnesoid X receptor in hereditary cholestasis associated to mutation in ATP8B1
about
Progressive familial intrahepatic cholestasisDifferential effects of progressive familial intrahepatic cholestasis type 1 and benign recurrent intrahepatic cholestasis type 1 mutations on canalicular localization of ATP8B1Bile acid metabolism and signalingHepatic farnesoid X-receptor isoforms α2 and α4 differentially modulate bile salt and lipoprotein metabolism in miceMissense mutations and single nucleotide polymorphisms in ABCB11 impair bile salt export pump processing and function or disrupt pre-messenger RNA splicingPhospholipase D2 mediates signaling by ATPase class I type 8B membrane 1Combined deletion of Fxr and Shp in mice induces Cyp17a1 and results in juvenile onset cholestasis.Progressive familial intrahepatic cholestasis.ATPase Class I Type 8B Member 1 and protein kinase C zeta induce the expression of the canalicular bile salt export pump in human hepatocytes.Two Cases of Progressive Familial Intrahepatic Cholestasis Type 2 Presenting with Severe Coagulopathy without Jaundice.ATP8B1 gene expression is driven by a housekeeping-like promoter independent of bile acids and farnesoid X receptor.Bile acid pool dynamics in progressive familial intrahepatic cholestasis with partial external bile diversion.ATP8B1 deficiency disrupts the bile canalicular membrane bilayer structure in hepatocytes, but FXR expression and activity are maintained.FXR: a target for cholestatic syndromes?Nuclear receptors as drug targets in cholestatic liver diseases.FXR signaling in metabolic disease.Compensatory induction of liver efflux transporters in response to ANIT-induced liver injury is impaired in FXR-null mice.The role of FXR in disorders of bile acid homeostasis.The membrane protein ATPase class I type 8B member 1 signals through protein kinase C zeta to activate the farnesoid X receptor.Effect of drug transporter pharmacogenetics on cholestasis.miR-33 controls the expression of biliary transporters, and mediates statin- and diet-induced hepatotoxicityKnockdown of ATP8B1 expression leads to specific downregulation of the bile acid sensor FXR in HepG2 cells: effect of the FXR agonist GW4064.Novel ATP8B1 mutation in an adult male with progressive familial intrahepatic cholestasis.Circadian dysregulation disrupts bile acid homeostasis.Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate.Hypoxia downregulates farnesoid X receptor via a hypoxia-inducible factor-independent but p38 mitogen-activated protein kinase-dependent pathway.Total internal biliary diversion during liver transplantation for type 1 progressive familial intrahepatic cholestasis: a novel approach.Functional analysis of ABCB4 mutations relates clinical outcomes of progressive familial intrahepatic cholestasis type 3 to the degree of MDR3 floppase activity.Heterozygous ABCB4 mutations in children with cholestatic liver disease.Omics-based responses induced by bosentan in human hepatoma HepaRG cell culturesBile salt export pump expression: can immunohistochemistry in isolation mislead?Alteration of MRP2 expression and the graft outcome after liver transplantation
P2860
Q21202893-6FE06AAF-A12A-4B15-A15A-CD8F9D3F514BQ24313001-A5091D71-A570-423F-9086-B45FF7A59E96Q27002873-A5EDF0EA-0429-4B8A-AAE3-BB791353C30FQ27320737-D9248188-3A1D-4430-AFF7-35F08CB22430Q28304808-D8D2343E-DC2B-4C40-8226-E04EBB2EC919Q28395197-2347A90C-2798-43FB-83EF-151675F7F9E9Q30433566-43A8D146-46C2-4D39-AE7B-A388207731E3Q33595403-BA0C18BB-0540-400F-9C23-DF64D1F1D234Q33643686-141183B4-642B-4B56-8BDC-DE6E1C3E4696Q33766117-8DF6B1F9-2D9D-44DC-9061-1F2CDC441F18Q34517090-6DEE1524-A9ED-46AD-B265-039741AD9D26Q35566171-5B05F3DA-782A-44FD-B819-9148E7D86275Q36228334-41055934-5D02-40E1-8200-5705AFCE2320Q36481061-3FC53DEE-D50A-4162-BC3B-85F345886569Q36786270-C851F324-F774-4EEE-9DB9-F4E534917E28Q37006382-87E4A5DC-54A1-4BAA-A287-93A4ACEBD251Q37227362-F0FCC651-7D6A-4FF4-8B42-CE3D147A38DDQ37300843-E3BAA704-715F-4055-8516-3EECA041C384Q37415558-B1CBF554-CA64-48B2-8503-A3FDCD778922Q38254730-6C25033F-D6B9-4E10-8C8D-3489C3102308Q38323834-9CDAF30D-2835-4F75-9795-D0C010A82CF8Q39881956-8D8939D4-A233-4429-868E-5D5EB91EB165Q42284483-9B48C4E8-6EB5-407F-AE42-40CDEF4491FFQ43066229-42D8CC1F-8EE1-4CD9-A522-3DB039DF9D6EQ43240642-892B559A-18EA-4068-AC24-5A6AC668E97EQ44950247-45B25883-6C28-4A6A-9E53-9419F443F2A1Q46917412-A8A5E069-87D4-441B-AE13-D352B1E11E36Q48327884-80248251-1F89-4533-8D8F-9381EE99F798Q53833514-B7405BD9-D8BA-49FC-86E6-F9A0F9E44464Q57119333-07228E2C-A5F3-408E-9DAA-939E196F9F11Q57152666-B028FAA5-8668-4939-9E8B-97EF6987BEA7Q58541981-6F093ED9-C95D-46D5-8D22-53CE844EAAF9
P2860
Reduced hepatic expression of farnesoid X receptor in hereditary cholestasis associated to mutation in ATP8B1
description
2004 nî lūn-bûn
@nan
2004 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
2004 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
2004年の論文
@ja
2004年論文
@yue
2004年論文
@zh-hant
2004年論文
@zh-hk
2004年論文
@zh-mo
2004年論文
@zh-tw
2004年论文
@wuu
name
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@ast
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en-gb
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@nl
type
label
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@ast
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en-gb
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@nl
prefLabel
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@ast
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en-gb
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@nl
P2093
P356
P1476
Reduced hepatic expression of ...... sociated to mutation in ATP8B1
@en
P2093
Angela De la Vega
Carmen Camarena
Eduardo López-Collazo
Elena Sánchez-Sabaté
Esteban Frauca
Javier Larrauri
Luis Alvarez
María C Díaz
Pablo Lapunzina
Paloma Jara
P304
P356
10.1093/HMG/DDH261
P407
P577
2004-08-18T00:00:00Z