Pages that link to "Q39770874"
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The following pages link to Reduced expression of ATP7B affected by Wilson disease-causing mutations is rescued by pharmacological folding chaperones 4-phenylbutyrate and curcumin (Q39770874):
Displaying 50 items.
- Clinical and experimental applications of sodium phenylbutyrate (Q24613511) (← links)
- Expression and in vivo rescue of human ABCC6 disease-causing mutants in mouse liver (Q27320511) (← links)
- The copper-transporting capacity of ATP7A mutants associated with Menkes disease is ameliorated by COMMD1 as a result of improved protein expression (Q28117183) (← links)
- Functional Rescue of Trafficking-Impaired ABCB4 Mutants by Chemical Chaperones (Q28550331) (← links)
- Identification of p38 MAPK and JNK as new targets for correction of Wilson disease-causing ATP7B mutants (Q28821905) (← links)
- The role of metal binding and phosphorylation domains in the regulation of cisplatin-induced trafficking of ATP7B. (Q33649147) (← links)
- Modifying factors and phenotypic diversity in Wilson's disease (Q33851780) (← links)
- Diverse functional properties of Wilson disease ATP7B variants (Q34125926) (← links)
- Clusterin (apolipoprotein J), a molecular chaperone that facilitates degradation of the copper-ATPases ATP7A and ATP7B. (Q34695507) (← links)
- Difference in stability of the N-domain underlies distinct intracellular properties of the E1064A and H1069Q mutants of copper-transporting ATPase ATP7B. (Q34963655) (← links)
- Critical roles for the COOH terminus of the Cu-ATPase ATP7B in protein stability, trans-Golgi network retention, copper sensing, and retrograde trafficking (Q35087086) (← links)
- Transmembrane helix 1 contributes to substrate translocation and protein stability of bile acid transporter SLC10A2. (Q35144856) (← links)
- Laser ablation inductively coupled plasma mass spectrometry imaging of metals in experimental and clinical Wilson's disease. (Q35391010) (← links)
- Clusterin and COMMD1 independently regulate degradation of the mammalian copper ATPases ATP7A and ATP7B (Q35709894) (← links)
- Advance in the pathogenesis and treatment of Wilson disease (Q36478429) (← links)
- The Menkes and Wilson disease genes counteract in copper toxicosis in Labrador retrievers: a new canine model for copper-metabolism disorders (Q36506584) (← links)
- Inside job: ligand-receptor pharmacology beneath the plasma membrane (Q36989788) (← links)
- Intestinal expression of metal transporters in Wilson's disease. (Q37302290) (← links)
- Targeting HSP90 and monoclonal protein trafficking modulates the unfolded protein response, chaperone regulation and apoptosis in myeloma cells (Q37423748) (← links)
- Disease-causing point-mutations in metal-binding domains of Wilson disease protein decrease stability and increase structural dynamics (Q37617523) (← links)
- Analysis of pseudoxanthoma elasticum-causing missense mutants of ABCC6 in vivo; pharmacological correction of the mislocalized proteins (Q37655761) (← links)
- Distinct phenotype of a Wilson disease mutation reveals a novel trafficking determinant in the copper transporter ATP7B. (Q37702034) (← links)
- Copper-induced hepatitis: the COMMD1 deficient dog as a translational animal model for human chronic hepatitis (Q37949685) (← links)
- Evolving perspectives in Wilson disease: diagnosis, treatment and monitoring (Q37956131) (← links)
- Population screening for Wilson's disease (Q38204501) (← links)
- Role of the transient receptor potential vanilloid 5 (TRPV5) protein N terminus in channel activity, tetramerization, and trafficking (Q38420343) (← links)
- Functional analysis and drug response to zinc and D-penicillamine in stable ATP7B mutant hepatic cell lines. (Q38774848) (← links)
- Quantification of ATP7B Protein in Dried Blood Spots by Peptide Immuno-SRM as a Potential Screen for Wilson's Disease. (Q39115525) (← links)
- Wilson disease protein ATP7B utilizes lysosomal exocytosis to maintain copper homeostasis. (Q41156563) (← links)
- Functional rescue of mutant ABCA1 proteins by sodium 4-phenylbutyrate (Q42010308) (← links)
- Characterization of ATP7A missense mutants suggests a correlation between intracellular trafficking and severity of Menkes disease (Q42189067) (← links)
- Wilson's disease: Prospective developments towards new therapies. (Q42345939) (← links)
- The genetics of Wilson disease (Q42380502) (← links)
- The six metal binding domains in human copper transporter, ATP7B: molecular biophysics and disease-causing mutations (Q46108156) (← links)
- A structural model of the copper ATPase ATP7B to facilitate analysis of Wilson disease-causing mutations and studies of the transport mechanism. (Q47630698) (← links)
- A systems biology approach reveals new endoplasmic reticulum-associated targets for the correction of the ATP7B mutant causing Wilson disease. (Q48673647) (← links)
- Targeted pharmacotherapy in progressive familial intrahepatic cholestasis type 2: Evidence for improvement of cholestasis with 4-phenylbutyrate (Q50438744) (← links)
- In Vivo Modeling of the Pathogenic Effect of Copper Transporter Mutations That Cause Menkes and Wilson Diseases, Motor Neuropathy, and Susceptibility to Alzheimer's Disease (Q50934293) (← links)
- Characterization of the most frequent ATP7B mutation causing Wilson disease in hepatocytes from patient induced pluripotent stem cells. (Q52314983) (← links)
- Novel compound heterozygote mutations in the ATP7B gene in an Iranian family with Wilson disease: a case report. (Q52655625) (← links)
- Functional Characterization of Novel ATP7B Variants for Diagnosis of Wilson Disease. (Q55002792) (← links)
- An αB-Crystallin Peptide Rescues Compartmentalization and Trafficking Response to Cu Overload of ATP7B-H1069Q, the Most Frequent Cause of Wilson Disease in the Caucasian Population (Q57116551) (← links)
- Curcumin Effect on Copper Transport in HepG2 Cells (Q57825815) (← links)
- Characteristics of neurological Wilson's disease with corpus callosum abnormalities (Q64067374) (← links)
- Chinese Herbal Medicine for Wilson's Disease: A Systematic Review and Meta-Analysis (Q64102549) (← links)
- Wilson disease-treatment perspectives. (Q64990860) (← links)
- Characterization of mutation spectrum and identification of novel mutations in ATP7B gene from a cohort of Wilson disease patients: Functional and therapeutic implications (Q91016642) (← links)
- Liver Expression of a MiniATP7B Gene Results in Long-Term Restoration of Copper Homeostasis in a Wilson Disease Model in Mice (Q91302378) (← links)
- Homozygous frame shift variant in ATP7B exon 1 leads to bypass of nonsense-mediated mRNA decay and to a protein capable of copper export (Q91361715) (← links)
- Age and Sex but Not ATP7B Genotype Effectively Influence the Clinical Phenotype of Wilson Disease (Q91558944) (← links)