2dwf: Difference between revisions

Latest revision as of 09:05, 6 November 2024

NMR structure of Mini-B, an N-terminal- C-terminal construct from human Surfactant Protein B (SP-B), in Sodium dodecyl sulfate (SDS) micelles

Structural highlights

2dwf is a 1 chain structure with sequence from Homo sapiens. Full experimental information is available from OCA. For a guided tour on the structure components use FirstGlance.
Method:	Solution NMR, 15 models
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

PSPB_HUMAN 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. 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.^[1] Genetic variations in SFTPB are a cause of susceptibility to respiratory distress syndrome in premature infants (RDS) [MIM:267450. 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'. Note=A variation Ile to Thr at position 131 influences the association between specific alleles of SFTPA1 and respiratory distress syndrome in premature infants.^[2]

Function

PSPB_HUMAN 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.

References

↑ Ballard PL, Nogee LM, Beers MF, Ballard RA, Planer BC, Polk L, deMello DE, Moxley MA, Longmore WJ. Partial deficiency of surfactant protein B in an infant with chronic lung disease. Pediatrics. 1995 Dec;96(6):1046-52. PMID:7491219
↑ Haataja R, Ramet M, Marttila R, Hallman M. Surfactant proteins A and B as interactive genetic determinants of neonatal respiratory distress syndrome. Hum Mol Genet. 2000 Nov 1;9(18):2751-60. PMID:11063734

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OCA

[1] Ballard PL, Nogee LM, Beers MF, Ballard RA, Planer BC, Polk L, deMello DE, Moxley MA, Longmore WJ. Partial deficiency of surfactant protein B in an infant with chronic lung disease. Pediatrics. 1995 Dec;96(6):1046-52. PMID:7491219

[2] Haataja R, Ramet M, Marttila R, Hallman M. Surfactant proteins A and B as interactive genetic determinants of neonatal respiratory distress syndrome. Hum Mol Genet. 2000 Nov 1;9(18):2751-60. PMID:11063734

[1]

[2]

@@ Line 1: / Line 1: @@
-[[Image:2dwf.jpg|left|200px]]<br /><applet load="2dwf" size="350" color="white" frame="true" align="right" spinBox="true"
-caption="2dwf" />
-'''NMR structure of Mini-B, an N-terminal- C-terminal construct from human Surfactant Protein B (SP-B), in Sodium dodecyl sulfate (SDS) micelles'''<br />
-==Disease==
+==NMR structure of Mini-B, an N-terminal- C-terminal construct from human Surfactant Protein B (SP-B), in Sodium dodecyl sulfate (SDS) micelles==
-Known disease associated with this structure: Surfactant metabolism dysfunction, pulmonary, 1 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=178640 178640]]
+<StructureSection load='2dwf' size='340' side='right'caption='[[2dwf]]' scene=''>
+== Structural highlights ==
-==About this Structure==
+<table><tr><td colspan='2'>[[2dwf]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2DWF OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2DWF FirstGlance]. <br>
-DWF is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/ ]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2DWF OCA].
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR, 15 models</td></tr>
-[[Category: Single protein]]
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=2dwf FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2dwf OCA], [https://pdbe.org/2dwf PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2dwf RCSB], [https://www.ebi.ac.uk/pdbsum/2dwf PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2dwf ProSAT]</span></td></tr>
-[[Category: Booth, V.]]
+</table>
-[[Category: Keough, K M.W.]]
+== Disease ==
-[[Category: Sarker, M.]]
+[https://www.uniprot.org/uniprot/PSPB_HUMAN PSPB_HUMAN] Defects in SFTPB are the cause of pulmonary surfactant metabolism dysfunction type 1 (SMDP1) [MIM:[https://omim.org/entry/265120 265120]; 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.<ref>PMID:7491219</ref>   Genetic variations in SFTPB are a cause of susceptibility to respiratory distress syndrome in premature infants (RDS) [MIM:[https://omim.org/entry/267450 267450]. 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'. Note=A variation Ile to Thr at position 131 influences the association between specific alleles of SFTPA1 and respiratory distress syndrome in premature infants.<ref>PMID:11063734</ref>
-[[Category: Waring, A J.]]
+== Function ==
-[[Category: lipid associated protein]]
+[https://www.uniprot.org/uniprot/PSPB_HUMAN PSPB_HUMAN] 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.
-[[Category: mini-b]]
+== References ==
-[[Category: sp-b]]
+<references/>
-[[Category: surfactant protein b]]
+__TOC__
+</StructureSection>
-''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 17:03:24 2008''
+[[Category: Homo sapiens]]
+[[Category: Large Structures]]
+[[Category: Booth V]]
+[[Category: Keough KMW]]
+[[Category: Sarker M]]
+[[Category: Waring AJ]]

2dwf: Difference between revisions

Latest revision as of 09:05, 6 November 2024

NMR structure of Mini-B, an N-terminal- C-terminal construct from human Surfactant Protein B (SP-B), in Sodium dodecyl sulfate (SDS) micelles

Structural highlights

Disease

Function

References

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