3hve

From Proteopedia

(Difference between revisions)

Revision as of 05:45, 27 June 2018

Structures of SPOP-Substrate Complexes: Insights into Molecular Architectures of BTB-Cul3 Ubiquitin Ligases: GigaxoninBTB/3-box

Structural highlights

3hve is a 2 chain structure with sequence from Human. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
NonStd Res:	,
Related:	3hqi, 3hql, 3hqm, 3htm, 3hu6, 3hsv
Gene:	GAN, GAN1, KLHL16 (HUMAN)
Resources:	FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT

Disease

[GAN_HUMAN] Defects in GAN are the cause of giant axonal neuropathy (GAN) [MIM: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.^[1] ^[2] ^[3] ^[4] ^[5] ^[6]

Function

[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.^[7] ^[8] ^[9] ^[10] ^[11]

Evolutionary Conservation

Check, as determined by ConSurfDB. You may read the explanation of the method and the full data available from ConSurf.

Publication Abstract from PubMed

In the largest E3 ligase subfamily, Cul3 binds a BTB domain, and an associated protein-interaction domain such as MATH recruits substrates for ubiquitination. Here, we present biochemical and structural analyses of the MATH-BTB protein, SPOP. We define a SPOP-binding consensus (SBC) and determine structures revealing recognition of SBCs from the phosphatase Puc, the transcriptional regulator Ci, and the chromatin component MacroH2A. We identify a dimeric SPOP-Cul3 assembly involving a conserved helical structure C-terminal of BTB domains, which we call "3-box" due to its facilitating Cul3 binding and its resemblance to F-/SOCS-boxes in other cullin-based E3s. Structural flexibility between the substrate-binding MATH and Cul3-binding BTB/3-box domains potentially allows a SPOP dimer to engage multiple SBCs found within a single substrate, such as Puc. These studies provide a molecular understanding of how MATH-BTB proteins recruit substrates to Cul3 and how their dimerization and conformational variability may facilitate avid interactions with diverse substrates.

Structures of SPOP-substrate complexes: insights into molecular architectures of BTB-Cul3 ubiquitin ligases.,Zhuang M, Calabrese MF, Liu J, Waddell MB, Nourse A, Hammel M, Miller DJ, Walden H, Duda DM, Seyedin SN, Hoggard T, Harper JW, White KP, Schulman BA Mol Cell. 2009 Oct 9;36(1):39-50. PMID:19818708^[12]

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.

References

↑ Wang W, Ding J, Allen E, Zhu P, Zhang L, Vogel H, Yang Y. Gigaxonin interacts with tubulin folding cofactor B and controls its degradation through the ubiquitin-proteasome pathway. Curr Biol. 2005 Nov 22;15(22):2050-5. PMID:16303566 doi:S0960-9822(05)01305-9
↑ Bomont P, Cavalier L, Blondeau F, Ben Hamida C, Belal S, Tazir M, Demir E, Topaloglu H, Korinthenberg R, Tuysuz B, Landrieu P, Hentati F, Koenig M. The gene encoding gigaxonin, a new member of the cytoskeletal BTB/kelch repeat family, is mutated in giant axonal neuropathy. Nat Genet. 2000 Nov;26(3):370-4. PMID:11062483 doi:10.1038/81701
↑ Kuhlenbaumer G, Young P, Oberwittler C, Hunermund G, Schirmacher A, Domschke K, Ringelstein B, Stogbauer F. Giant axonal neuropathy (GAN): case report and two novel mutations in the gigaxonin gene. Neurology. 2002 Apr 23;58(8):1273-6. PMID:11971098
↑ Bomont P, Ioos C, Yalcinkaya C, Korinthenberg R, Vallat JM, Assami S, Munnich A, Chabrol B, Kurlemann G, Tazir M, Koenig M. Identification of seven novel mutations in the GAN gene. Hum Mutat. 2003 Apr;21(4):446. PMID:12655563 doi:10.1002/humu.9122
↑ Houlden H, Groves M, Miedzybrodzka Z, Roper H, Willis T, Winer J, Cole G, Reilly MM. New mutations, genotype phenotype studies and manifesting carriers in giant axonal neuropathy. J Neurol Neurosurg Psychiatry. 2007 Nov;78(11):1267-70. Epub 2007 Jun 19. PMID:17578852 doi:10.1136/jnnp.2007.118968
↑ Koop O, Schirmacher A, Nelis E, Timmerman V, De Jonghe P, Ringelstein B, Rasic VM, Evrard P, Gartner J, Claeys KG, Appenzeller S, Rautenstrauss B, Huhne K, Ramos-Arroyo MA, Worle H, Moilanen JS, Hammans S, Kuhlenbaumer G. Genotype-phenotype analysis in patients with giant axonal neuropathy (GAN). Neuromuscul Disord. 2007 Aug;17(8):624-30. Epub 2007 Jun 22. PMID:17587580 doi:S0960-8966(07)00114-9
↑ Ding J, Liu JJ, Kowal AS, Nardine T, Bhattacharya P, Lee A, Yang Y. Microtubule-associated protein 1B: a neuronal binding partner for gigaxonin. J Cell Biol. 2002 Aug 5;158(3):427-33. Epub 2002 Jul 29. PMID:12147674 doi:10.1083/jcb.200202055
↑ Furukawa M, He YJ, Borchers C, Xiong Y. Targeting of protein ubiquitination by BTB-Cullin 3-Roc1 ubiquitin ligases. Nat Cell Biol. 2003 Nov;5(11):1001-7. Epub 2003 Oct 5. PMID:14528312 doi:10.1038/ncb1056
↑ Wang W, Ding J, Allen E, Zhu P, Zhang L, Vogel H, Yang Y. Gigaxonin interacts with tubulin folding cofactor B and controls its degradation through the ubiquitin-proteasome pathway. Curr Biol. 2005 Nov 22;15(22):2050-5. PMID:16303566 doi:S0960-9822(05)01305-9
↑ Zhang DD, Lo SC, Sun Z, Habib GM, Lieberman MW, Hannink M. Ubiquitination of Keap1, a BTB-Kelch substrate adaptor protein for Cul3, targets Keap1 for degradation by a proteasome-independent pathway. J Biol Chem. 2005 Aug 26;280(34):30091-9. Epub 2005 Jun 27. PMID:15983046 doi:10.1074/jbc.M501279200
↑ Allen E, Ding J, Wang W, Pramanik S, Chou J, Yau V, Yang Y. Gigaxonin-controlled degradation of MAP1B light chain is critical to neuronal survival. Nature. 2005 Nov 10;438(7065):224-8. Epub 2005 Oct 16. PMID:16227972 doi:nature04256
↑ Zhuang M, Calabrese MF, Liu J, Waddell MB, Nourse A, Hammel M, Miller DJ, Walden H, Duda DM, Seyedin SN, Hoggard T, Harper JW, White KP, Schulman BA. Structures of SPOP-substrate complexes: insights into molecular architectures of BTB-Cul3 ubiquitin ligases. Mol Cell. 2009 Oct 9;36(1):39-50. PMID:19818708 doi:10.1016/j.molcel.2009.09.022

1 Structural highlights
2 Disease
3 Function
4 Evolutionary Conservation
5 Publication Abstract from PubMed
6 See Also
7 References

Proteopedia Page Contributors and Editors (what is this?)

OCA

Retrieved from "http://52.214.119.220/wiki/index.php/3hve"

@@ Line 1: / Line 1: @@
 ==Structures of SPOP-Substrate Complexes: Insights into Molecular Architectures of BTB-Cul3 Ubiquitin Ligases: GigaxoninBTB/3-box==
 <StructureSection load='3hve' size='340' side='right' caption='[[3hve]], [[Resolution|resolution]] 2.80&Aring;' scene=''>
@@ Line 6: / Line 7: @@
 <tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[3hqi|3hqi]], [[3hql|3hql]], [[3hqm|3hqm]], [[3htm|3htm]], [[3hu6|3hu6]], [[3hsv|3hsv]]</td></tr>
 <tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">GAN, GAN1, KLHL16 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
-<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3hve FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3hve OCA], [http://pdbe.org/3hve PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3hve RCSB], [http://www.ebi.ac.uk/pdbsum/3hve PDBsum]</span></td></tr>
+<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3hve FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3hve OCA], [http://pdbe.org/3hve PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3hve RCSB], [http://www.ebi.ac.uk/pdbsum/3hve PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3hve ProSAT]</span></td></tr>
 </table>
 == Disease ==
@@ Line 16: / Line 17: @@
 Check<jmol>
   <jmolCheckbox>
-    <scriptWhenChecked>select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/hv/3hve_consurf.spt"</scriptWhenChecked>
+    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/hv/3hve_consurf.spt"</scriptWhenChecked>
     <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
     <text>to colour the structure by Evolutionary Conservation</text>
@@ Line 41: / Line 42: @@
 [[Category: Schulman, B A]]
 [[Category: Zhuang, M]]
+[[Category: Cytoplasm]]
 [[Category: Cytoskeleton]]
 [[Category: Disease mutation]]
@@ Line 48: / Line 50: @@
 [[Category: Neurodegeneration]]
 [[Category: Phosphoprotein]]
+[[Category: Polymorphism]]
 [[Category: Protein binding]]
 [[Category: Ubiquitin]]
+[[Category: Ubl conjugation]]
 [[Category: Ubl conjugation pathway]]

3hve

From Proteopedia

Revision as of 05:45, 27 June 2018

Structures of SPOP-Substrate Complexes: Insights into Molecular Architectures of BTB-Cul3 Ubiquitin Ligases: GigaxoninBTB/3-box

Structural highlights

Disease

Function

Evolutionary Conservation

Publication Abstract from PubMed

See Also

References

Contents

Proteopedia Page Contributors and Editors (what is this?)

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