1egj
From Proteopedia
m (Protected "1egj" [edit=sysop:move=sysop]) |
|||
Line 1: | Line 1: | ||
- | [[Image:1egj.png|left|200px]] | ||
- | |||
{{STRUCTURE_1egj| PDB=1egj | SCENE= }} | {{STRUCTURE_1egj| PDB=1egj | SCENE= }} | ||
+ | ===DOMAIN 4 OF THE BETA COMMON CHAIN IN COMPLEX WITH AN ANTIBODY=== | ||
+ | {{ABSTRACT_PUBMED_10753826}} | ||
- | == | + | ==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> | ||
+ | ==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== | ||
Line 10: | Line 13: | ||
==Reference== | ==Reference== | ||
- | <ref group="xtra">PMID:010753826</ref><references group="xtra"/> | + | <ref group="xtra">PMID:010753826</ref><references group="xtra"/><references/> |
[[Category: Homo sapiens]] | [[Category: Homo sapiens]] | ||
[[Category: Mus musculus]] | [[Category: Mus musculus]] |
Revision as of 20:39, 24 March 2013
Contents |
DOMAIN 4 OF THE BETA COMMON CHAIN IN COMPLEX WITH AN ANTIBODY
Template:ABSTRACT PUBMED 10753826
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
1egj is a 3 chain structure with sequence from Homo sapiens and Mus musculus. Full crystallographic information is available from OCA.
Reference
- Rossjohn J, McKinstry WJ, Woodcock JM, McClure BJ, Hercus TR, Parker MW, Lopez AF, Bagley CJ. Structure of the activation domain of the GM-CSF/IL-3/IL-5 receptor common beta-chain bound to an antagonist. Blood. 2000 Apr 15;95(8):2491-8. PMID:10753826
- ↑ 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