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C-terminally mutated tubby protein accumulates in aggresomes

Title
C-terminally mutated tubby protein accumulates in aggresomes
Authors
Kim, SunshinSung, Ho JinLee, Ji WonKim, Yun HeeOh, Yong-SeokYoon, Kyong-AhHeo, KyunSuh, Pann-Ghill
DGIST Authors
Oh, Yong-Seok
Issue Date
2017
Citation
BMB Reports, 50(1), 37-42
Type
Article
Article Type
Article
Keywords
Adaptor Proteins, Signal TransducingAggresomeAnimalAnimalsBiosynthesisBrainBrainC57Bl MouseCell Line, TumorCell NucleusCell NucleusCercopithecus AethiopsChlorocebus AethiopsCOS Cell LineCOS CellsDefectsDiseasesGene FamilyGeneticsIdentificationMetabolismMiceMice, Inbred C57BLMisfoldingMouseMouseMutantsMutationMutationNeuroblastomaNeuroblastomaObesityObesityObesityProtein AggregateProtein AggregatesProtein Degradation End ProductProtein Degradation End ProductsProtein FoldingProtein FoldingRecessive MutationsRetinitis PigmentosaRNA SplicingRNA SplicingSignal Transducing Adaptor ProteinTub Protein, MouseTubbyTULP1Tumor Cell Line
ISSN
1976-6696
Abstract
The tubby protein (Tub), a putative transcription factor, plays important roles in the maintenance and function of neuronal cells. A splicing defect-causing mutation in the 3'-end of the tubby gene, which is predicted to disrupt the carboxy-terminal region of the Tub protein, causes maturity-onset obesity, blindness, and deafness in mice. Although this pathological Tub mutation leads to a loss of function, the precise mechanism has not yet been investigated. Here, we found that the mutant Tub proteins were mostly localized to puncta found in the perinuclear region and that the C-terminus was important for its solubility. Immunocytochemical analysis revealed that puncta of mutant Tub co-localized with the aggresome. Moreover, whereas wild-type Tub was translocated to the nucleus by extracellular signaling, the mutant forms failed to undergo such translocation. Taken together, our results suggest that the malfunctions of the Tub mutant are caused by its misfolding and subsequent localization to aggresomes. © 2017 by the The Korean Society for Biochemistry and Molecular Biology.
URI
http://hdl.handle.net/20.500.11750/4254
DOI
10.5483/BMBRep.2017.50.1.140
Publisher
The Biochemical Society of the Republic of Korea
Related Researcher
Files:
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Collection:
Brain and Cognitive SciencesETC1. Journal Articles


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