6mun

<StructureSection load='6mun' size='340' side='right'caption='[[6mun]], [[NMR_Ensembles_of_Models | 10 NMR models]]' scene=''>

<table><tr><td colspan='2'>[[6mun]] is a 3 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6MUN OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6MUN FirstGlance]. <br>

</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[1yx4|1yx4]], [[1yx5|1yx5]], [[1yx6|1yx6]], [[1j8c|1j8c]], [[2kde|2kde]], [[2kdf|2kdf]]</td></tr>

<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PSMD4, MCB1 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN]), UBQLN2, N4BP4, PLIC2, HRIHFB2157 ([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=6mun FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6mun OCA], [http://pdbe.org/6mun PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6mun RCSB], [http://www.ebi.ac.uk/pdbsum/6mun PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6mun ProSAT]</span></td></tr>

== Disease ==

[[http://www.uniprot.org/uniprot/UBQL2_HUMAN UBQL2_HUMAN]] Defects in UBQLN2 are the cause of amyotrophic lateral sclerosis type 15 with or without frontotemporal dementia (ALS15) [MIM:[http://omim.org/entry/300857 300857]]. A neurodegenerative disorder affecting upper motor neurons in the brain and lower motor neurons in the brain stem and spinal cord, resulting in fatal paralysis. Sensory abnormalities are absent. The pathologic hallmarks of the disease include pallor of the corticospinal tract due to loss of motor neurons, presence of ubiquitin-positive inclusions within surviving motor neurons, and deposition of pathologic aggregates. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of the cases. Patients with ALS15 may develop frontotemporal dementia.<ref>PMID:21857683</ref> <ref>PMID:22892309</ref> <ref>PMID:22717235</ref> <ref>PMID:22560112</ref>

[[http://www.uniprot.org/uniprot/PSMD4_HUMAN PSMD4_HUMAN]] Binds and presumably selects ubiquitin-conjugates for destruction. Displays selectivity for longer polyubiquitin chains. Modulates intestinal fluid secretion. [[http://www.uniprot.org/uniprot/UBQL2_HUMAN UBQL2_HUMAN]] Increases the half-life of proteins destined to be degraded by the proteasome; may modulate proteasome-mediated protein degradation.<ref>PMID:10983987</ref>

 

 

== References ==

<references/>

[[Category: Human]]

[[Category: Chen, X]]

[[Category: Walters, K J]]

[[Category: Structural protein]]