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Suh, Pann-Ghill
BioSignal Network Lab (BSN)
Research Interests
  • Signal transduction, cancer, metabolism, phospholipase C

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C-terminally mutated tubby protein accumulates in aggresomes

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Title
C-terminally mutated tubby protein accumulates in aggresomes
Author
Kim, SunshinSung, Ho JinLee, Ji WonKim, Yun HeeOh, Yong-SeokYoon, Kyong-AhHeo, KyunSuh, Pann-Ghill
Keywords
Aggresome; Misfolding; Mutation; Obesity; Tubby
Issue Date
201701
Publisher
KOREAN SOCIETY BIOCHEMISTRY & MOLECULAR BIOLOGY
Citation
BMB REPORTS, v.50, no.1, pp.37 - 42
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.
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DOI
http://dx.doi.org/10.5483/BMBRep.2017.50.1.140
ISSN
1976-6696
Appears in Collections:
SLS_Journal Papers
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