
TRIMRINGfingerproteinTRIM2,aubiquitinligase,ishighlyexpressedinthenervoussystem.Itbindstoneurofilamentlightsubunit(NF-L)andregulatesNF-Lubiquitination.DeficienciesinTRIM2increasesNF-LlevelinaxonsandNF-L-filledaxonalswellingsincerebellum,retina,spinalcord,andcerebralcortex.
Image:TRIM2(dilution:6.3µg/ml)stainingofparaffinembeddedHumanCortex.SteamedantigenretrievalwithcitratebufferpH6,AP-staining.Notecytoplasmstaininginneuronalcellbodies.Protocol ondatasheet.
Thesedeficiencieseventuallyleadtoneurodegeneration,tremorsandataxia.ItisproposedthatTRIM2hasneuroprotectionproperties.