Sequence in raw or FASTA format:
SFTPB surfactant protein B [Homo sapiens (human)]
|Entrez Gene ID||6439|
|Full Name||surfactant protein B|
|Synonyms||PSP-B, SFTB3, SFTP3, SMDP1, SP-B|
|General protein information||
|Organism||Homo sapiens (human)|
|Summary||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].|
Surfactant metabolism dysfunction, pulmonary, 1, 265120 (3)
|Homo sapiens (human)||SFTPB||NP_000533.3|
|Pan troglodytes (chimpanzee)||SFTPB||XP_001168379.2|
|Macaca mulatta (Rhesus monkey)||SFTPB||XP_001089841.1|
|Canis lupus familiaris (dog)||SFTPB||XP_005630540.1|
|Bos taurus (cattle)||SFTPB||NP_001068779.1|
|Mus musculus (house mouse)||Sftpb||NP_001269000.1|
|Rattus norvegicus (Norway rat)||Sftpb||NP_620197.1|
|Xenopus (Silurana) tropicalis (western clawed frog)||sftpb||XP_002932589.2|
|GO:0006665||sphingolipid metabolic process||IEA|
|GO:0007585||respiratory gaseous exchange||IEA|
|PDB||2M1T, 1RG3, 1RG4, 2M0H, 1SSZ, 1DFW, 1KMR, 2DWF, 2JOU|
What is the normal function of the SFTPB gene?
The SFTPB gene provides instructions for making a protein called surfactant protein-B (SP-B). This protein is one of four proteins (each produced from a different gene) in surfactant, a mixture of certain fats (called phospholipids) and proteins that lines the lung tissue and makes breathing easy. Without normal surfactant, the tissue surrounding the air sacs in the lungs (the alveoli) sticks together after exhalation (because of a force called surface tension), causing the alveoli to collapse. As a result, filling the lungs with air on each breath becomes very difficult, and the delivery of oxygen to the body is impaired. Surfactant lowers surface tension, easing breathing and avoiding lung collapse. The SP-B protein helps spread the surfactant across the surface of the lung tissue, aiding in the surface tension-lowering property of surfactant.
The phospholipids and proteins that make up surfactant are packaged in cellular structures known as lamellar bodies, which are found in specialized lung cells. The surfactant proteins must go through several processing steps to mature and become functional; some of these steps occur in lamellar bodies. The SP-B protein plays a role in the formation of lamellar bodies and, thus, affects the processing of a surfactant protein called surfactant protein-C (SP-C).
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