PubRank

Leo W J Klomp

Author PubWeight™ 67.89‹?›

Top papers

Rank Title Journal Year PubWeight™‹?›
1 The gene product Murr1 restricts HIV-1 replication in resting CD4+ lymphocytes. Nature 2003 2.63
2 Farnesoid X receptor activation inhibits inflammation and preserves the intestinal barrier in inflammatory bowel disease. Gut 2011 2.57
3 Nasobiliary drainage induces long-lasting remission in benign recurrent intrahepatic cholestasis. Hepatology 2006 2.37
4 Mutations in VPS33B, encoding a regulator of SNARE-dependent membrane fusion, cause arthrogryposis-renal dysfunction-cholestasis (ARC) syndrome. Nat Genet 2004 2.04
5 Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin. Hepatology 2009 1.92
6 A novel role for XIAP in copper homeostasis through regulation of MURR1. EMBO J 2003 1.90
7 Folding defects in P-type ATP 8B1 associated with hereditary cholestasis are ameliorated by 4-phenylbutyrate. Hepatology 2010 1.76
8 Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11. Gastroenterology 2004 1.60
9 The H1069Q mutation in ATP7B is associated with late and neurologic presentation in Wilson disease: results of a meta-analysis. J Hepatol 2004 1.49
10 Human copper transporter 2 is localized in late endosomes and lysosomes and facilitates cellular copper uptake. Biochem J 2007 1.47
11 Two mass-spectrometric techniques for quantifying serine enantiomers and glycine in cerebrospinal fluid: potential confounders and age-dependent ranges. Clin Chem 2008 1.45
12 Cisplatin rapidly down-regulates its own influx transporter hCTR1 in cultured human ovarian carcinoma cells. Clin Cancer Res 2004 1.44
13 Familial cholestasis: progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy. Best Pract Res Clin Gastroenterol 2010 1.35
14 ATP8B1 is essential for maintaining normal hearing. Proc Natl Acad Sci U S A 2009 1.30
15 Biochemical characterization and subcellular localization of human copper transporter 1 (hCTR1). Biochem J 2002 1.29
16 The ubiquitously expressed MURR1 protein is absent in canine copper toxicosis. J Hepatol 2003 1.29
17 Characterization of COMMD protein-protein interactions in NF-kappaB signalling. Biochem J 2006 1.27
18 Distinct Wilson's disease mutations in ATP7B are associated with enhanced binding to COMMD1 and reduced stability of ATP7B. Gastroenterology 2007 1.23
19 Heteromeric interactions required for abundance and subcellular localization of human CDC50 proteins and class 1 P4-ATPases. J Biol Chem 2010 1.20
20 Bile acids and their nuclear receptor FXR: Relevance for hepatobiliary and gastrointestinal disease. Biochim Biophys Acta 2010 1.20
21 Transcription factor Sp1 plays an important role in the regulation of copper homeostasis in mammalian cells. Mol Pharmacol 2008 1.16
22 Liver disease associated with canalicular transport defects: current and future therapies. J Hepatol 2009 1.13
23 Clusterin facilitates COMMD1 and I-kappaB degradation to enhance NF-kappaB activity in prostate cancer cells. Mol Cancer Res 2010 1.12
24 D-amino acids in the central nervous system in health and disease. Mol Genet Metab 2005 1.12
25 Elevated glutathione levels confer cellular sensitization to cisplatin toxicity by up-regulation of copper transporter hCtr1. Mol Pharmacol 2008 1.09
26 The N-terminus of the human copper transporter 1 (hCTR1) is localized extracellularly, and interacts with itself. Biochem J 2003 1.09
27 New developments in the regulation of intestinal copper absorption. Nutr Rev 2009 1.09
28 Variation of linker length in ratiometric fluorescent sensor proteins allows rational tuning of Zn(II) affinity in the picomolar to femtomolar range. J Am Chem Soc 2007 1.06
29 Copper-dependent protein-protein interactions studied by yeast two-hybrid analysis. Biochem Biophys Res Commun 2004 1.05
30 Fic1 is expressed at apical membranes of different epithelial cells in the digestive tract and is induced in the small intestine during postnatal development of mice. Pediatr Res 2004 1.04
31 Activation of bile salt nuclear receptor FXR is repressed by pro-inflammatory cytokines activating NF-κB signaling in the intestine. Biochim Biophys Acta 2011 1.03
32 A flippase-independent function of ATP8B1, the protein affected in familial intrahepatic cholestasis type 1, is required for apical protein expression and microvillus formation in polarized epithelial cells. Hepatology 2010 1.03
33 Serine-deficiency syndromes. Curr Opin Neurol 2004 1.03
34 Raised hepatic bile acid concentrations during pregnancy in mice are associated with reduced farnesoid X receptor function. Hepatology 2010 1.02
35 Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum. J Hepatol 2002 1.01
36 Nuclear-cytosolic transport of COMMD1 regulates NF-kappaB and HIF-1 activity. Traffic 2009 0.99
37 Molecular regulation of copper excretion in the liver. Proc Nutr Soc 2004 0.98
38 COMMD1 expression is controlled by critical residues that determine XIAP binding. Biochem J 2009 0.97
39 Gene expression profiling of liver cells after copper overload in vivo and in vitro reveals new copper-regulated genes. J Biol Inorg Chem 2007 0.96
40 Liver-specific Commd1 knockout mice are susceptible to hepatic copper accumulation. PLoS One 2011 0.94
41 ATOX1: a novel copper-responsive transcription factor in mammals? Int J Biochem Cell Biol 2008 0.94
42 D-serine in the developing human central nervous system. Ann Neurol 2006 0.93
43 Posttranslational regulation of copper transporters. J Biol Inorg Chem 2009 0.92
44 Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B. J Biol Chem 2011 0.90
45 The copper-transporting capacity of ATP7A mutants associated with Menkes disease is ameliorated by COMMD1 as a result of improved protein expression. Cell Mol Life Sci 2011 0.89
46 Monitoring bile acid transport in single living cells using a genetically encoded Förster resonance energy transfer sensor. Hepatology 2013 0.89
47 Cu,Zn superoxide dismutase maturation and activity are regulated by COMMD1. J Biol Chem 2010 0.89
48 Biochemical and cellular functions of P4 ATPases. Biochem J 2010 0.85
49 Ratiometric fluorescent sensor proteins with subnanomolar affinity for Zn(II) based on copper chaperone domains. J Am Chem Soc 2006 0.83
50 Clusterin (apolipoprotein J), a molecular chaperone that facilitates degradation of the copper-ATPases ATP7A and ATP7B. J Biol Chem 2011 0.81
51 Role of transition metals in the pathogenesis of amyotrophic lateral sclerosis. Biochem Soc Trans 2008 0.81
52 Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1. Kidney Int 2004 0.81
53 Extensive changes in liver gene expression induced by hereditary tyrosinemia type I are not normalized by treatment with 2-(2-nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC). J Hepatol 2003 0.80
54 D-serine influences synaptogenesis in a p19 cell model. JIMD Rep 2012 0.76
55 Idiopathic hypoalbuminemia explained by reduced synthesis rate and an increased catabolic rate. Clin Biochem 2002 0.76
56 Kidneys of mice with hereditary tyrosinemia type I are extremely sensitive to cytotoxicity. Pediatr Res 2006 0.76
57 Cerebrospinal fluid D-serine and glycine concentrations are unaltered and unaffected by olanzapine therapy in male schizophrenic patients. Eur Neuropsychopharmacol 2008 0.75
58 Increased concentrations of both NMDA receptor co-agonists D-serine and glycine in global ischemia: a potential novel treatment target for perinatal asphyxia. Amino Acids 2011 0.75