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A gene encoding a liver-specific ABC transporter is mutated in progressive familial intrahepatic cholestasis
- S. Strautnieks, L. Bull, +16 authors Richard Thompson
- Biology, Medicine
- Nature Genetics
- 1 November 1998
Data provide evidence that SPGP is the human bile salt export pump (BSEP), and the product of the orthologous rat gene has been shown to be an effective bile acid transporter in vitro. Expand
The wide spectrum of multidrug resistance 3 deficiency: from neonatal cholestasis to cirrhosis of adulthood.
At least one third of the patients with a progressive familial intrahepatic cholestasis type 3 phenotype have a proven defect of the multidrug resistance 3 gene (MDR3), which should also be considered in adult liver diseases. Expand
Native umbilical cord matrix stem cells express hepatic markers and differentiate into hepatocyte-like cells.
It is concluded that UCMSCs, with a newly demonstrated endodermic differentiation potential, might be an alternative source for liver-directed cell therapies. Expand
A role for autophagy during hepatic stellate cell activation.
- L. Thoen, E. Guimarães, +4 authors L. V. van Grunsven
- Biology, Medicine
- Journal of hepatology
- 1 April 2012
The demonstration of partly inhibition of in vitro HSC activation after treatment with an autophagy inhibitor unveils a potential new therapeutic strategy for liver fibrosis. Expand
Hepatocanalicular bile salt export pump deficiency in patients with progressive familial intrahepatic cholestasis.
Investigation of expression of the bile salt export pump in liver samples from patients with a PFIC phenotype and correlated this with BSEP gene mutations shows a close correlation between BSEp gene mutations and canalicular B SEP expression. Expand
Immunogenicity and tolerability of recombinant serogroup B meningococcal vaccine administered with or without routine infant vaccinations according to different immunization schedules: a randomized…
A 4CMenB vaccine is immunogenic against reference strains when administered with routine vaccines at 2, 4, and 6 years of age, producing minimal interference with the response to routine infant vaccinations. Expand
Recombinant gp350 vaccine for infectious mononucleosis: a phase 2, randomized, double-blind, placebo-controlled trial to evaluate the safety, immunogenicity, and efficacy of an Epstein-Barr virus…
- E. Sokal, K. Hoppenbrouwers, +7 authors M. Denis
- The Journal of infectious diseases
- 15 December 2007
These data support the clinical feasibility of using an EBV vaccine to prevent infectious mononucleosis and there were no concerns regarding the safety or reactogenicity of the gp350/AS04 vaccine. Expand
Severe bile salt export pump deficiency: 82 different ABCB11 mutations in 109 families.
With this study, >100 ABCB11 mutations are now identified and close surveillance of BSEP-deficient patients retaining their native liver, particularly those carrying 2 null mutations, is essential. Expand
Management of chronic hepatitis B in childhood: ESPGHAN clinical practice guidelines: consensus of an expert panel on behalf of the European Society of Pediatric Gastroenterology, Hepatology and…
Safe and effective antiviral therapies are available in adults, but few are labeled for the use in children, and an accurate selection of whom to treat and the identification of the right timing for treatment are needed to optimize response and reduce the risk of antiviral resistance. Expand
Interferon alfa therapy for chronic hepatitis B in children: a multinational randomized controlled trial.
In children with chronic hepatitis B, INF-alpha promotes loss of viral replication markers and surface antigen and improves aminotransferases and histology and reduces dose in 24% of children. Expand