功能： disease:Defects in SFTPB are the cause of pulmonary surfactant metabolism dysfunction type 1 (SMDP1) [MIM:265120]; also called pulmonary alveolar proteinosis due to surfactant protein B deficiency. Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular non-specific interstitial pneumonitis (NSIP).,function: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.,miscellaneous:Pulmonary surfactant consists of 90% lipid and 10% protein. There are 4 surfactant-associated proteins: 2 collagenous, carbohydrate-binding glycoproteins (SP-A and SP-D) and 2 small hydrophobic proteins (SP-B and SP-C).,polymorphism:Genetic variation at position 131 may influence the association between specific alleles of SFTPA1 and respiratory distress syndrome in premature infants (RDS) [MIM:267450].,similarity:Contains 1 saposin A-type domain.,similarity:Contains 3 saposin B-type domains.,subunit:Homodimer; disulfide-linked.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Secreted, extracellular space, surface film.
功能： disease:Defects in SFTPB are the cause of pulmonary surfactant metabolism dysfunction type 1 (SMDP1) [MIM:265120]; also called pulmonary alveolar proteinosis due to surfactant protein B deficiency. Inborn errors of pulmonary surfactant metabolism are genetically heterogeneous disorders resulting in severe respiratory insufficiency or failure in full-term neonates or infants. These disorders are associated with various pathologic entities, including pulmonary alveolar proteinosis (PAP), desquamative interstitial pneumonitis (DIP), or cellular non-specific interstitial pneumonitis (NSIP).,function: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.,miscellaneous:Pulmonary surfactant consists of 90% lipid and 10% protein. There are 4 surfactant-associated proteins: 2 collagenous, carbohydrate-binding glycoproteins (SP-A and SP-D) and 2 small hydrophobic proteins (SP-B and SP-C).,polymorphism:Genetic variation at position 131 may influence the association between specific alleles of SFTPA1 and respiratory distress syndrome in premature infants (RDS) [MIM:267450].,similarity:Contains 1 saposin A-type domain.,similarity:Contains 3 saposin B-type domains.,subunit:Homodimer; disulfide-linked.,
相关产品： RS0001,RS0002,YM3028,YM3029
细胞定位： Secreted, extracellular space, surface film.
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