4blb: Difference between revisions

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{{STRUCTURE_4blb|  PDB=4blb  |  SCENE=  }}  
{{STRUCTURE_4blb|  PDB=4blb  |  SCENE=  }}  
===Crystal structure of a human Suppressor of fused (SUFU)-GLI1p complex===
===Crystal structure of a human Suppressor of fused (SUFU)-GLI1p complex===
{{ABSTRACT_PUBMED_10559945}}
{{ABSTRACT_PUBMED_16459298}}
 
==Disease==
[[http://www.uniprot.org/uniprot/GLI3_HUMAN GLI3_HUMAN]] Postaxial polydactyly type B, unilateral;Postaxial polydactyly type A, unilateral;Pallister-Hall syndrome;Postaxial polydactyly type A, bilateral;Polysyndactyly, bilateral;Polysyndactyly, unilateral;Greig cephalopolysyndactyly syndrome;Acrocallosal syndrome;Postaxial polydactyly type B, bilateral. The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry.  The disease is caused by mutations affecting the gene represented in this entry.


==Function==
==Function==
[[http://www.uniprot.org/uniprot/MALE_ECOLI MALE_ECOLI]] Involved in the high-affinity maltose membrane transport system MalEFGK. Initial receptor for the active transport of and chemotaxis toward maltooligosaccharides. [[http://www.uniprot.org/uniprot/GLI3_HUMAN GLI3_HUMAN]] Has a dual function as a transcriptional activator and a repressor of the sonic hedgehog (Shh) pathway, and plays a role in limb development. The full-length GLI3 form (GLI3FL) after phosphorylation and nuclear translocation, acts as an activator (GLI3A) while GLI3R, its C-terminally truncated form, acts as a repressor. A proper balance between the GLI3 activator and the repressor GLI3R, rather than the repressor gradient itself or the activator/repressor ratio gradient, specifies limb digit number and identity. In concert with TRPS1, plays a role in regulating the size of the zone of distal chondrocytes, in restricting the zone of PTHLH expression in distal cells and in activating chondrocyte proliferation. Binds to the minimal GLI-consensus sequence 5'-GGGTGGTC-3'.<ref>PMID:10693759</ref> <ref>PMID:11238441</ref> <ref>PMID:17764085</ref>   
[[http://www.uniprot.org/uniprot/MALE_ECOLI MALE_ECOLI]] Involved in the high-affinity maltose membrane transport system MalEFGK. Initial receptor for the active transport of and chemotaxis toward maltooligosaccharides. [[http://www.uniprot.org/uniprot/GLI1_HUMAN GLI1_HUMAN]] Acts as a transcriptional activator. May regulate the transcription of specific genes during normal development. May play a role in craniofacial development and digital development, as well as development of the central nervous system and gastrointestinal tract. Mediates SHH signaling and thus cell proliferation and differentiation.<ref>PMID:10806483</ref> <ref>PMID:11238441</ref>   


==About this Structure==
==About this Structure==
[[4blb]] is a 8 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4BLB OCA].  
[[4blb]] is a 8 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4BLB OCA].  


==Reference==
==Reference==
<ref group="xtra">PMID:010559945</ref><references group="xtra"/><references/>
<ref group="xtra">PMID:016459298</ref><references group="xtra"/><references/>
[[Category: Human]]
[[Category: Cherry, A L.]]
[[Category: Cherry, A L.]]
[[Category: Finta, C.]]
[[Category: Finta, C.]]
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