42 results on '"Klomp, Leo W.J."'
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2. Data from Clusterin Facilitates COMMD1 and I-κB Degradation to Enhance NF-κB Activity in Prostate Cancer Cells
3. Familial cholestasis: Progressive familial intrahepatic cholestasis, benign recurrent intrahepatic cholestasis and intrahepatic cholestasis of pregnancy
4. Cerebrospinal fluid d-serine and glycine concentrations are unaltered and unaffected by olanzapine therapy in male schizophrenic patients
5. New developments in the regulation of intestinal copper absorption
6. ATP8B1 is essential for maintaining normal hearing
7. Ratiometric fluorescent sensor proteins with subnanomolar affinity for Zn(II) based on copper chaperone domains
8. 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
9. Renal proximal tubular cells acquire resistance to cell death stimuli in mice with hereditary tyrosinemia type 1
10. Benign recurrent intrahepatic cholestasis type 2 is caused by mutations in ABCB11
11. The H1069Q mutation in ATP7B is associated with late and neurologic presentation in Wilson disease: results of a meta-analysis
12. FIC1 Disease: A Spectrum of Intrahepatic Cholestatic Disorders
13. 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)
14. The ubiquitously expressed MURR1 protein is absent in canine copper toxicosis
15. Aceruloplasminemia: an inherited neurodegenerative disease with impairment of iron homeostasis
16. Ceruloplasmin Gene Expression in the Murine Central Nervous System
17. Variation of linker length in ratiometric fluorescent sensor proteins allows rational tuning of Zn(II) affinity in the picomolar to femtomolar range
18. Clusterin and COMMD1 Independently Regulate Degradation of the Mammalian Copper ATPases ATP7A and ATP7B
19. Activation of bile salt nuclear receptor FXR is repressed by pro-inflammatory cytokines activating NF-κB signaling in the intestine
20. Clusterin (Apolipoprotein J), a Molecular Chaperone That Facilitates Degradation of the Copper-ATPases ATP7A and ATP7B
21. Heteromeric Interactions Required for Abundance and Subcellular Localization of Human CDC50 Proteins and Class 1 P4-ATPases
22. Cu,Zn Superoxide Dismutase Maturation and Activity Are Regulated by COMMD1
23. Bile acids and their nuclear receptor FXR: Relevance for hepatobiliary and gastrointestinal disease
24. Liver disease associated with canalicular transport defects: Current and future therapies
25. Clusterin Facilitates COMMD1 and I-κB Degradation to Enhance NF-κB Activity in Prostate Cancer Cells
26. ATOX1: A novel copper-responsive transcription factor in mammals?
27. Distinct Wilson’s Disease Mutations in ATP7B Are Associated With Enhanced Binding to COMMD1 and Reduced Stability of ATP7B
28. d-Amino acids in the central nervous system in health and disease
29. Copper-dependent protein–protein interactions studied by yeast two-hybrid analysis
30. The N-terminus of the human copper transporter 1 (hCTR1) is localized extracellularly, and interacts with itself
31. Idiopathic hypoalbuminemia explained by reduced synthesis rate and an increased catabolic rate
32. Biochemical characterization and subcellular localization of human copper transporter 1 (hCTR1)
33. Benign recurrent intrahepatic cholestasis progressing to progressive familial intrahepatic cholestasis: low GGT cholestasis is a clinical continuum
34. FIC1, the protein affected in two forms of hereditary cholestasis, is localized in the cholangiocyte and the canalicular membrane of the hepatocyte
35. Molecular Characterization of 3-Phosphoglycerate Dehydrogenase Deficiency—a Neurometabolic Disorder Associated with Reduced L-Serine Biosynthesis
36. Structure, Expression, and Chromosomal Localization of the Mouse Atox1 Gene
37. A gene encoding a P-type ATPase mutated in two forms of hereditary cholestasis
38. The Copper Chaperone for Superoxide Dismutase
39. Identification and Functional Expression of HAH1, a Novel Human Gene Involved in Copper Homeostasis
40. The gene product Murr1 restricts HIV-1 replication in resting CD4+ lymphocytes.
41. Kidneys of Mice With Hereditary Tyrosinemia Type I Are Extremely Sensitive to Cytotoxicity
42. 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
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