Biomaterial Database

Cystic fibrosis: to ion transport and beyond. 2010

A Bush, and J Davies

UI	MeSH Term	Description	Entries
D003550	Cystic Fibrosis	An autosomal recessive genetic disease of the EXOCRINE GLANDS. It is caused by mutations in the gene encoding the CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR expressed in several organs including the LUNG, the PANCREAS, the BILIARY SYSTEM, and the SWEAT GLANDS. Cystic fibrosis is characterized by epithelial secretory dysfunction associated with ductal obstruction resulting in AIRWAY OBSTRUCTION; chronic RESPIRATORY INFECTIONS; PANCREATIC INSUFFICIENCY; maldigestion; salt depletion; and HEAT PROSTRATION.	Mucoviscidosis,Cystic Fibrosis of Pancreas,Fibrocystic Disease of Pancreas,Pancreatic Cystic Fibrosis,Pulmonary Cystic Fibrosis,Cystic Fibrosis, Pancreatic,Cystic Fibrosis, Pulmonary,Fibrosis, Cystic,Pancreas Fibrocystic Disease,Pancreas Fibrocystic Diseases
D004195	Disease Models, Animal	Naturally-occurring or experimentally-induced animal diseases with pathological processes analogous to human diseases.	Animal Disease Model,Animal Disease Models,Disease Model, Animal
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000818	Animals	Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA.	Animal,Metazoa,Animalia
D053503	Epithelial Sodium Channels	Sodium channels found on salt-reabsorbing EPITHELIAL CELLS that line the distal NEPHRON; the distal COLON; SALIVARY DUCTS; SWEAT GLANDS; and the LUNG. They are AMILORIDE-sensitive and play a critical role in the control of sodium balance, BLOOD VOLUME, and BLOOD PRESSURE.	Epithelial Sodium Channel,Epithelial Sodium Ion Channels,ENaC (Epithelial Na+ Channel),ENaC alpha,ENaC beta,ENaC delta,ENaC gamma,Epithelial Amiloride-Sensitive Sodium Channel,Epithelial Sodium Channel, alpha Subunit,Epithelial Sodium Channel, beta Subunit,Epithelial Sodium Channel, delta Subunit,Epithelial Sodium Channel, gamma Subunit,SCNN1 alpha Subunit,SCNN1 beta Subunit,SCNN1 delta Subunit,SCNN1 gamma Subunit,Sodium Channel, Nonvoltage-gated 1 Protein, alpha Subunit,Sodium Channel, Nonvoltage-gated 1 Protein, beta Subunit,Sodium Channel, Nonvoltage-gated 1 Protein, delta Subunit,Sodium Channel, Nonvoltage-gated 1 Protein, gamma Subunit,Epithelial Amiloride Sensitive Sodium Channel,Sodium Channel, Epithelial,Sodium Channels, Epithelial,alpha Subunit, SCNN1
D019005	Cystic Fibrosis Transmembrane Conductance Regulator	A chloride channel that regulates secretion in many exocrine tissues. Abnormalities in the CFTR gene have been shown to cause cystic fibrosis. (Hum Genet 1994;93(4):364-8)	CFTR Protein,Chloride channels, ATP-gated CFTR,Chloride channels, ATP gated CFTR,Protein, CFTR