1gh7
From Proteopedia
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- | [[Image:1gh7.png|left|200px]] | ||
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{{STRUCTURE_1gh7| PDB=1gh7 | SCENE= }} | {{STRUCTURE_1gh7| PDB=1gh7 | SCENE= }} | ||
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===CRYSTAL STRUCTURE OF THE COMPLETE EXTRACELLULAR DOMAIN OF THE BETA-COMMON RECEPTOR OF IL-3, IL-5, AND GM-CSF=== | ===CRYSTAL STRUCTURE OF THE COMPLETE EXTRACELLULAR DOMAIN OF THE BETA-COMMON RECEPTOR OF IL-3, IL-5, AND GM-CSF=== | ||
+ | {{ABSTRACT_PUBMED_11207369}} | ||
- | + | ==Disease== | |
+ | [[http://www.uniprot.org/uniprot/IL3RB_HUMAN IL3RB_HUMAN]] Defects in CSF2RB are the cause of pulmonary surfactant metabolism dysfunction type 5 (SMDP5) [MIM:[http://omim.org/entry/614370 614370]]. SMDP5 is 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:21075760</ref> | ||
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+ | ==Function== | ||
+ | [[http://www.uniprot.org/uniprot/IL3RB_HUMAN IL3RB_HUMAN]] High affinity receptor for interleukin-3, interleukin-5 and granulocyte-macrophage colony-stimulating factor. | ||
==About this Structure== | ==About this Structure== | ||
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==Reference== | ==Reference== | ||
- | <ref group="xtra">PMID:011207369</ref><references group="xtra"/> | + | <ref group="xtra">PMID:011207369</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Carr, P D.]] | [[Category: Carr, P D.]] |
Revision as of 04:26, 25 March 2013
Contents |
CRYSTAL STRUCTURE OF THE COMPLETE EXTRACELLULAR DOMAIN OF THE BETA-COMMON RECEPTOR OF IL-3, IL-5, AND GM-CSF
Template:ABSTRACT PUBMED 11207369
Disease
[IL3RB_HUMAN] Defects in CSF2RB are the cause of pulmonary surfactant metabolism dysfunction type 5 (SMDP5) [MIM:614370]. SMDP5 is 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]
Function
[IL3RB_HUMAN] High affinity receptor for interleukin-3, interleukin-5 and granulocyte-macrophage colony-stimulating factor.
About this Structure
1gh7 is a 2 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.
Reference
- Carr PD, Gustin SE, Church AP, Murphy JM, Ford SC, Mann DA, Woltring DM, Walker I, Ollis DL, Young IG. Structure of the complete extracellular domain of the common beta subunit of the human GM-CSF, IL-3, and IL-5 receptors reveals a novel dimer configuration. Cell. 2001 Jan 26;104(2):291-300. PMID:11207369
- ↑ Tanaka T, Motoi N, Tsuchihashi Y, Tazawa R, Kaneko C, Nei T, Yamamoto T, Hayashi T, Tagawa T, Nagayasu T, Kuribayashi F, Ariyoshi K, Nakata K, Morimoto K. Adult-onset hereditary pulmonary alveolar proteinosis caused by a single-base deletion in CSF2RB. J Med Genet. 2011 Mar;48(3):205-9. doi: 10.1136/jmg.2010.082586. Epub 2010 Nov, 12. PMID:21075760 doi:10.1136/jmg.2010.082586