2mak: Difference between revisions
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{{STRUCTURE_2mak| PDB=2mak | SCENE= }} | |||
===Solution structure of the STIM1 CC1-CC2 homodimer in complex with two Orai1 C-terminal domains.=== | |||
{{ABSTRACT_PUBMED_24351972}} | |||
==Disease== | |||
[[http://www.uniprot.org/uniprot/STIM1_HUMAN STIM1_HUMAN]] Defects in STIM1 are the cause of immune dysfunction with T-cell inactivation due to calcium entry defect type 2 (IDTICED2) [MIM:[http://omim.org/entry/612783 612783]]. IDTICED2 is an immune disorder characterized by recurrent infections, impaired T-cell activation and proliferative response, decreased T-cell production of cytokines, lymphadenopathy, and normal lymphocytes counts and serum immunoglobulin levels. Additional features include thrombocytopenia, autoimmune hemolytic anemia, non-progressive myopathy, partial iris hypoplasia, hepatosplenomegaly and defective enamel dentition.<ref>PMID:19420366</ref> [[http://www.uniprot.org/uniprot/CRCM1_HUMAN CRCM1_HUMAN]] Combined immunodeficiency due to ORAI1 deficiency. The disease is caused by mutations affecting the gene represented in this entry. | |||
==Function== | |||
[[http://www.uniprot.org/uniprot/STIM1_HUMAN STIM1_HUMAN]] Plays a role in mediating store-operated Ca(2+) entry (SOCE), a Ca(2+) influx following depletion of intracellular Ca(2+) stores. Acts as Ca(2+) sensor in the endoplasmic reticulum via its EF-hand domain. Upon Ca(2+) depletion, translocates from the endoplasmic reticulum to the plasma membrane where it activates the Ca(2+) release-activated Ca(2+) (CRAC) channel subunit, TMEM142A/ORAI1.<ref>PMID:9377559</ref> <ref>PMID:16005298</ref> <ref>PMID:15866891</ref> <ref>PMID:16208375</ref> <ref>PMID:16807233</ref> <ref>PMID:16766533</ref> <ref>PMID:16733527</ref> <ref>PMID:16537481</ref> <ref>PMID:22464749</ref> [[http://www.uniprot.org/uniprot/CRCM1_HUMAN CRCM1_HUMAN]] Ca(2+) release-activated Ca(2+) (CRAC) channel subunit which mediates Ca(2+) influx following depletion of intracellular Ca(2+) stores and channel activation by the Ca(2+) sensor, STIM1. CRAC channels are the main pathway for Ca(2+) influx in T-cells and promote the immune response to pathogens by activating the transcription factor NFAT.<ref>PMID:16766533</ref> <ref>PMID:16807233</ref> <ref>PMID:16733527</ref> <ref>PMID:16645049</ref> <ref>PMID:16582901</ref> | |||
==About this Structure== | |||
[[2mak]] is a 4 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2MAK OCA]. | |||
==Reference== | |||
<ref group="xtra">PMID:024351972</ref><references group="xtra"/><references/> | |||
[[Category: Ikura, M.]] | |||
[[Category: Stathopulos, P B.]] | |||
[[Category: Coiled-coil]] | |||
[[Category: Orai1]] | |||
[[Category: Orai1 c-terminal domain]] | |||
[[Category: Signaling protein-transport protein complex]] | |||
[[Category: Stim1]] | |||
[[Category: Transport protein]] |