HADIDA-RUAH SARA,MILLER MARK,ZHOU JINGLAN,BEAR BRIAN,GROOTENHUIS PETER
申请号：
NZ61609709
公开号：
NZ616097A
申请日:
2009.03.25
申请国别(地区):
NZ
年份:
2015
代理人:
摘要：
The disclosure relates to a pyridine-clopropane-carboxamide derivatives as exemplified by the formulae shown herein, and a pharmaceutically acceptable salt thereof, processes for their preparation, compositions comprising said compounds and uses thereof. Said compound is useful as modulators of ATP-Binding Cassette (ABE) transporters or fragments thereof, including Cystic Fibrosis Transmembrane Conductance Regulator (&rdquo；CFTR&rdquo；), as such are useful in the treatment of condition, disease, or disorder selected from cystic fibrosis, hereditary emphysema, hereditary hemochromatosis, coagulation-fibrinolysis deficiencies, protein C deficiency, Type 1 hereditary angioedema, lipid processing deficiencies, familial hypercholesterolemia, Type 1 chylomicronemia, abetalipoproteinemia, lysosomal storage diseases, I-cell disease/pseudo-Hurler, mucopolysaccharidoses, Sandhof/Tay-Sachs, Crigler-Najjar type II, polyendocrinopathy/hyperinsulemia, diabetes mellitus, laron dwarfism, myleoperoxidase deficiency, primary hypoparathyroidism, melanoma, glycanosis CDG type 1, hereditary emphysema, congenital hyperthyroidism, osteogenesis imperfecta, hereditary ypofibrinogenemia, ACT deficiency, diabetes insipidus (di), neurophyseal di, neprogenic DI, Charcot-Marie Tooth syndrome, Perlizaeus-Merzbacher disease, Alzheimer&rsquo；s disease, Parkinson&rsquo；s disease, amyotrophic lateral sclerosis, progressive supranuclear plasy, Pick&rsquo；s disease, Huntington, spinocerebullar ataxia type I, spinal and bulbar muscular atrophy, dentatorubal pallidoluysian, myotonic dystrophy, as well as spongiform encephalopathies, hereditary Creutzfeldt-Jakob disease, Fabry disease, Straussler-Scheinker syndrome, COPD, dry-eye disease, and Sjögren&rsquo；s disease.
