467 results on '"Kai, Hirofumi"'
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2. Combinatorial screening for therapeutics in ATTRv amyloidosis identifies naphthoquinone analogues as TTR-selective amyloid disruptors
3. Metformin suppresses epithelial sodium channel hyperactivation and its associated phenotypes in a mouse model of obstructive lung diseases
4. NanoLuc reporters identify COL4A5 nonsense mutations susceptible to drug-induced stop codon readthrough
5. The 2019 and 2021 International Workshops on Alport Syndrome
6. Type IV Collagen Variants in CKD: Performance of Computational Predictions for Identifying Pathogenic Variants
7. Intrapopulation analysis of longitudinal lifespan in Caenorhabditis elegans identifies W09D10.4 as a novel AMPK-associated healthspan shortening factor
8. Trimerization and Genotype–Phenotype Correlation of COL4A5 Mutants in Alport Syndrome
9. TALENs Facilitate Single-step Seamless SDF Correction of F508del CFTR in Airway Epithelial Submucosal Gland Cell-derived CF-iPSCs
10. Metformin ameliorates the severity of experimental Alport syndrome
11. A cell-based high-throughput screening method to directly examine transthyretin amyloid fibril formation at neutral pH
12. Auto-measure emphysematous parameters and pathophysiological gene expression profiles in experimental mouse models of acute and chronic obstructive pulmonary diseases
13. T3 Intratracheal Therapy Alleviates Pulmonary Pathology in an Elastase-Induced Emphysema-Dominant COPD Mouse Model
14. 6-Formylindolo[3,2-b]Carbazole Accelerates Skin Wound Healing via Activation of ERK, but Not Aryl Hydrocarbon Receptor
15. Mild electrical stimulation with heat shock attenuates renal pathology in adriamycin-induced nephrotic syndrome mouse model
16. Development of an exon skipping therapy for X-linked Alport syndrome with truncating variants in COL4A5
17. The DsbA-L gene is associated with respiratory function of the elderly via its adiponectin multimeric or antioxidant properties
18. Melinjo seed extract increases adiponectin multimerization in physiological and pathological conditions
19. T 3 Intratracheal Therapy Alleviates Pulmonary Pathology in an Elastase-Induced Emphysema-Dominant COPD Mouse Model.
20. CyclosporinA Derivative as Therapeutic Candidate for Alport Syndrome by Inducing Mutant Type IV Collagen Secretion
21. Endoplasmic Reticulum (ER) Stress Induces Sirtuin 1 (SIRT1) Expression via the PI3K-Akt-GSK3β Signaling Pathway and Promotes Hepatocellular Injury
22. Pre-emptive Quality Control Protects the ER from Protein Overload via the Proximity of ERAD Components and SRP
23. Cross-talk between PKA-Cβ and p65 mediates synergistic induction of PDE4B by roflumilast and NTHi
24. Correction: The 2019 and 2021 International workshops on Alport syndrome
25. Mild Electrical Stimulation with Heat Shock Reduces Visceral Adiposity and Improves Metabolic Abnormalities in Subjects with Metabolic Syndrome or Type 2 Diabetes: Randomized Crossover Trials
26. The Transcription Factor MEF/Elf4 Is Dually Modulated by p53-MDM2 Axis and MEF-MDM2 Autoregulatory Mechanism
27. Lipopolysaccharide Decreases Single Immunoglobulin Interleukin-1 Receptor-related Molecule (SIGIRR) Expression by Suppressing Specificity Protein 1 (Sp1) via the Toll-like Receptor 4 (TLR4)-p38 Pathway in Monocytes and Neutrophils
28. Evaluation of Three-Dimensional Cultured HepG2 Cells in a Nano Culture Plate System: an In Vitro Human Model of Acetaminophen Hepatotoxicity
29. Free radical scavenging, α-glucosidase inhibitory and lipase inhibitory activities of eighteen Sudanese medicinal plants
30. Mild Electrical Stimulation at 0.1-ms Pulse Width Induces p53 Protein Phosphorylation and G2 Arrest in Human Epithelial Cells
31. α1-Acid Glycoprotein Up-regulates CD163 via TLR4/CD14 Protein Pathway: POSSIBLE PROTECTION AGAINST HEMOLYSIS-INDUCED OXIDATIVE STRESS
32. STT3B-Dependent Posttranslational N-Glycosylation as a Surveillance System for Secretory Protein
33. Phosphodiesterase 4B Mediates Extracellular Signal-regulated Kinase-dependent Up-regulation of Mucin MUC5AC Protein by Streptococcus pneumoniae by Inhibiting cAMP-protein Kinase A-dependent MKP-1 Phosphatase Pathway
34. Synergism Between Interleukin (IL)-17 and Toll-like Receptor 2 and 4 Signals to Induce IL-8 Expression in Cystic Fibrosis Airway Epithelial Cells
35. A Splice Switch in SIGIRR Causes a Defect of IL-37-Dependent Anti-Inflammatory Activity in Cystic Fibrosis Airway Epithelial Cells
36. CFTR-deficiency renders mice highly susceptible to cutaneous symptoms during mite infestation
37. Glucose Uptake in Rat Skeletal Muscle L6 Cells Is Increased by Low-Intensity Electrical Current Through the Activation of the Phosphatidylinositol-3-OH Kinase (PI-3K) / Akt Pathway
38. Novel Keap1-Nrf2 Protein-Protein Interaction Inhibitor UBE-1099 Ameliorates Progressive Phenotype in Alport Syndrome Mouse Model
39. Seamless Gene Correction in the Human Cystic Fibrosis Transmembrane Conductance Regulator Locus by Vector Replacement and Vector Insertion Events
40. Seamless Gene Correction in the Human Cystic Fibrosis Transmembrane Conductance Regulator Locus by Vector Replacement and Vector Insertion Events
41. The Endoplasmic Reticulum-associated Degradation of Transthyretin Variants Is Negatively Regulated by BiP in Mammalian Cells
42. Tumor Suppressor Cylindromatosis Acts as a Negative Regulator for Streptococcus pneumoniae-induced NFAT Signaling
43. Mild Electrical Stimulation Increases Ubiquitinated Proteins and Hsp72 in A549 Cells via Attenuation of Proteasomal Degradation
44. Role of calnexin in the ER quality control and productive folding of CFTR; differential effect of calnexin knockout on wild-type and ΔF508 CFTR
45. Phosphatidic acid metabolism regulates the intracellular trafficking and retrotranslocation of CFTR
46. Non-thermal and transient thermal effects of burst 100 MHz sinusoidal electric fields on apoptotic activity in HeLa cells
47. Clinical trial recommendations for potential Alport syndrome therapies
48. Endoplasmic reticulum quality control regulates the fate of transthyretin variants in the cell
49. Differential mode of cholesterol inclusion with 2‐hydroxypropyl‐cyclodextrins increases safety margin in treatment of Niemann‐Pick disease type C
50. Activation of heat shock response improves biomarkers of NAFLD in patients with metabolic diseases
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