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Protein Page:
SFTPB (human)

Overview
SFTPB Pulmonary surfactant-associated proteins promote alveolar stability by lowering the surface tension at the air- liquid interface in the peripheral air spaces. SP-B increases the collapse pressure of palmitic acid to nearly 70 millinewtons per meter. Defects in SFTPB are the cause of pulmonary surfactant metabolism dysfunction type 1 (SMDP1); also called pulmonary alveolar proteinosis due to surfactant protein B deficiency. A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid- Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress. Genetic variations in SFTPB are a cause of susceptibility to respiratory distress syndrome in premature infants (RDS). RDS is a lung disease affecting usually premature newborn infants. It is characterized by deficient gas exchange, diffuse atelectasis, high-permeability lung edema and fibrin-rich alveolar deposits called 'hyaline membranes'. A variation Ile to Thr at position 131 influences the association between specific alleles of SFTPA1 and respiratory distress syndrome in premature infants. Note: This description may include information from UniProtKB.
Protein type: Secreted, signal peptide; Secreted
Cellular Component: extracellular space; lysosome
Biological Process: organ morphogenesis; sphingolipid metabolic process; respiratory gaseous exchange
Reference #:  P07988 (UniProtKB)
Alt. Names/Synonyms: P07988; PSP-B; Pulmonary surfactant-associated protein B, 18kD; SFTB3; SFTP3; SFTPB; SMDP1; SP-B; surfactant protein B; surfactant, pulmonary-associated protein B
Gene Symbols: SFTPB
Molecular weight: 42,117 Da
Basal Isoelectric point: 5.27  Predict pI for various phosphorylation states
Select Structure to View Below

SFTPB

Protein Structure Not Found.


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