NP_000533.3
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
42,117 Da
NCBI Official Full Name
pulmonary surfactant-associated protein B
NCBI Official Synonym Full Names
surfactant protein B
NCBI Official Synonym Symbols
SP-B; PSP-B; SFTB3; SFTP3; SMDP1 [Similar Products]
NCBI Protein Information
pulmonary surfactant-associated protein B; 6 kDa protein; 18 kDa pulmonary-surfactant protein; pulmonary surfactant-associated proteolipid SPL(Phe)
UniProt Protein Name
Pulmonary surfactant-associated protein B
UniProt Synonym Protein Names
18 kDa pulmonary-surfactant protein; 6 kDa protein; Pulmonary surfactant-associated proteolipid SPL(Phe)
UniProt Synonym Gene Names
UniProt Entry Name
PSPB_HUMAN
NCBI Summary for SPB
This gene encodes the pulmonary-associated surfactant protein B (SPB), an amphipathic surfactant protein essential for lung function and homeostasis after birth. Pulmonary surfactant is a surface-active lipoprotein complex composed of 90% lipids and 10% proteins which include plasma proteins and apolipoproteins SPA, SPB, SPC and SPD. The surfactant is secreted by the alveolar cells of the lung and maintains the stability of pulmonary tissue by reducing the surface tension of fluids that coat the lung. The SPB enhances the rate of spreading and increases the stability of surfactant monolayers in vitro. Multiple mutations in this gene have been identified, which cause pulmonary surfactant metabolism dysfunction type 1, also called pulmonary alveolar proteinosis due to surfactant protein B deficiency, and are associated with fatal respiratory distress in the neonatal period. Alternatively spliced transcript variants encoding the same protein have been identified.[provided by RefSeq, Feb 2010]
UniProt Comments for SPB
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.
Protein type: Secreted, signal peptide; Secreted
Chromosomal Location of Human Ortholog: 2p12-p11.2
Cellular Component: extracellular space; lysosome
Biological Process: organ morphogenesis; sphingolipid metabolic process; respiratory gaseous exchange
Disease: Surfactant Metabolism Dysfunction, Pulmonary, 1
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Diseases associated with SPB elisa kit
Organs/Tissues associated with SPB elisa kit
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