2laz: Difference between revisions

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[[Image:2laz.jpg|left|200px]]
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{{STRUCTURE_2laz|  PDB=2laz  |  SCENE=  }}  
{{STRUCTURE_2laz|  PDB=2laz  |  SCENE=  }}  
===Structure of the first WW domain of human Smurf1 in complex with a mono-phosphorylated human Smad1 derived peptide===
===Structure of the first WW domain of human Smurf1 in complex with a mono-phosphorylated human Smad1 derived peptide===
{{ABSTRACT_PUBMED_21685363}}


==Disease==
[[http://www.uniprot.org/uniprot/SMAD1_HUMAN SMAD1_HUMAN]] Defects in SMAD1 may be a cause of primary pulmonary hypertension (PPH1) [MIM:[http://omim.org/entry/178600 178600]]. A rare disorder characterized by plexiform lesions of proliferating endothelial cells in pulmonary arterioles. The lesions lead to elevated pulmonary arterial pression, right ventricular failure, and death. The disease can occur from infancy throughout life and it has a mean age at onset of 36 years. Penetrance is reduced. Although familial PPH1 is rare, cases secondary to known etiologies are more common and include those associated with the appetite-suppressant drugs.<ref>PMID:21898662</ref>


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==Function==
The line below this paragraph, {{ABSTRACT_PUBMED_21685363}}, adds the Publication Abstract to the page
[[http://www.uniprot.org/uniprot/SMUF1_HUMAN SMUF1_HUMAN]] E3 ubiquitin-protein ligase that acts as a negative regulator of BMP signaling pathway. Mediates ubiquitination and degradation of SMAD1 and SMAD5, 2 receptor-regulated SMADs specific for the BMP pathway. Promotes ubiquitination and subsequent proteasomal degradation of TRAF family members and RHOA.<ref>PMID:10458166</ref><ref>PMID:19937093</ref><ref>PMID:21402695</ref> [[http://www.uniprot.org/uniprot/SMAD1_HUMAN SMAD1_HUMAN]] Transcriptional modulator activated by BMP (bone morphogenetic proteins) type 1 receptor kinase. SMAD1 is a receptor-regulated SMAD (R-SMAD). SMAD1/OAZ1/PSMB4 complex mediates the degradation of the CREBBP/EP300 repressor SNIP1.<ref>PMID:12097147</ref>  
(as it appears on PubMed at http://www.pubmed.gov), where 21685363 is the PubMed ID number.
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{{ABSTRACT_PUBMED_21685363}}


==About this Structure==
==About this Structure==
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==Reference==
==Reference==
<ref group="xtra">PMID:021685363</ref><references group="xtra"/>
<ref group="xtra">PMID:021685363</ref><references group="xtra"/><references/>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
[[Category: Aragon, E.]]
[[Category: Aragon, E.]]
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