2ppi: Difference between revisions

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~~[[Image:2ppi.png|left|200px]]~~

===Structure of the BTB (Tramtrack and Bric a brac) domain of human Gigaxonin===

===~~Structure~~ of the ~~BTB (Tramtrack~~ and ~~Bric a brac) domain~~ of ~~human Gigaxonin===~~

==Disease==

[[http://www.uniprot.org/uniprot/GAN_HUMAN GAN_HUMAN]] Defects in GAN are the cause of giant axonal neuropathy (GAN) [MIM:[http://omim.org/entry/256850 256850]]. GAN is a severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. It is characterized by neurofilament accumulation, leading to segmental distention of axons.<ref>PMID:16303566</ref><ref>PMID:11062483</ref><ref>PMID:11971098</ref><ref>PMID:12655563</ref><ref>PMID:17578852</ref><ref>PMID:17587580</ref>

==Function==

[[http://www.uniprot.org/uniprot/GAN_HUMAN GAN_HUMAN]] Probable cytoskeletal component that directly or indirectly plays an important role in neurofilament architecture. Substrate-specific adapter of an E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Controls degradation of TBCB. Controls degradation of MAP1B and MAP1S, and is critical for neuronal maintenance and survival.<ref>PMID:12147674</ref><ref>PMID:14528312</ref><ref>PMID:16303566</ref><ref>PMID:15983046</ref><ref>PMID:16227972</ref>

==About this Structure==

[[2ppi]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2PPI OCA].

==Reference==

[[Category: Homo sapiens]]

[[Category: Amos, A.]]

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-[[Image:2ppi.png|left|200px]]
 {{STRUCTURE_2ppi|  PDB=2ppi  |  SCENE=  }}
+===Structure of the BTB (Tramtrack and Bric a brac) domain of human Gigaxonin===
-===Structure of the BTB (Tramtrack and Bric a brac) domain of human Gigaxonin===
+==Disease==
+[[http://www.uniprot.org/uniprot/GAN_HUMAN GAN_HUMAN]] Defects in GAN are the cause of giant axonal neuropathy (GAN) [MIM:[http://omim.org/entry/256850 256850]]. GAN is a severe autosomal recessive sensorimotor neuropathy affecting both the peripheral nerves and the central nervous system. It is characterized by neurofilament accumulation, leading to segmental distention of axons.<ref>PMID:16303566</ref><ref>PMID:11062483</ref><ref>PMID:11971098</ref><ref>PMID:12655563</ref><ref>PMID:17578852</ref><ref>PMID:17587580</ref>
+==Function==
+[[http://www.uniprot.org/uniprot/GAN_HUMAN GAN_HUMAN]] Probable cytoskeletal component that directly or indirectly plays an important role in neurofilament architecture. Substrate-specific adapter of an E3 ubiquitin-protein ligase complex which mediates the ubiquitination and subsequent proteasomal degradation of target proteins. Controls degradation of TBCB. Controls degradation of MAP1B and MAP1S, and is critical for neuronal maintenance and survival.<ref>PMID:12147674</ref><ref>PMID:14528312</ref><ref>PMID:16303566</ref><ref>PMID:15983046</ref><ref>PMID:16227972</ref>
 ==About this Structure==
 [[2ppi]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2PPI OCA].
+==Reference==
+<references group="xtra"/><references/>
 [[Category: Homo sapiens]]
 [[Category: Amos, A.]]