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pulmonary surfactant-associated protein A1; PSP-A; PSPA; SP-A; SP-A1; 35 kD pulmonary surfactant-associated protein; Alveolar proteinosis protein; collectin-4 (SFTPA1, COLEC4, PSAP, SFTP1, SFTPA, SFTPA1B)

Function: 1) binds to surfactant phospholipids (Ca+2-dependent) 2) contributes to lower the surface tension at the air-liquid interface in the alveoli 3) essential for normal respiration Structure: 1) oligomeric complex of 6 set of homotrimers 2) contains 1 C-type lectin domain. - contains 1 collagen-like domain Compartment: - secreted, extracellular space, extracellular matrix, surface film Polymorphism: - at least 5 allelic variants of SFTPA1 are known: 6A, 6A(2), 6A(3), 6A(4) & 6A(5) Pathology: - genetic variations in SFTPA1 are a cause of a) susceptibility to idiopathic pulmonary fibrosis b) susceptibility to neonatal respiratory distress syndrome - association between SFTPA1 alleles & neonatal RDS is dependent on a variation Ile to Thr at position 131 in SFTPB

Related

pulmonary surfactant-associated protein A2 (SP-A2, SP-A, PSP-A, PSPA, alveolar proteinosis protein, 35 kD pulmonary surfactant-associated protein, SFTPA2, PSAP, SFTP1, SFTPA)

General

glycoprotein secreted protein

Properties

SIZE: entity length = 248 aa MW = 26 kD COMPARTMENT: extracellular compartment MOTIF: signal sequence {1-20} cysteine residue {C26} MODIFICATION: cysteine residue {CINTERCHAIN (PROBABLE} Collagen-like {28-100} C-type lectin {132-248} MOTIF: cysteine residue {C155} MODIFICATION: cysteine residue {C246} N-glycosylation site {N207} cysteine residue {C224} MODIFICATION: cysteine residue {C238} cysteine residue {C238} MODIFICATION: cysteine residue {C224} cysteine residue {C246} MODIFICATION: cysteine residue {C155}

Database Correlations

OMIM correlations MORBIDMAP 178630 UniProt Q8IWL2 PFAM correlations Entrez Gene 653509 Kegg hsa:653509

References

  1. UniProt :accession Q8IWL2
  2. SeattleSNPs http://pga.gs.washington.edu/data/sftpa1/
  3. Functional glycomics gateway - glycan binding Note: Pulmonary surfactant protein SP-A1 http://www.functionalglycomics.org/glycomics/GBPServlet?&operationtype=view&cbpId=cbp_hum_Ctlect_232