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C-terminally mutated tubby protein accumulates in aggresomes
- C-terminally mutated tubby protein accumulates in aggresomes
- Kim, Sunshin; Sung, Ho Jin; Lee, Ji Won; Kim, Yun Hee; Oh, Yong-Seok; Yoon, Kyong-Ah; Heo, Kyun; Suh, Pann-Ghill
- DGIST Authors
- Oh, Yong-Seok
- Issue Date
- BMB Reports, 50(1), 37-42
- Article Type
- Adaptor Proteins, Signal Transducing; Aggresome; Animal; Animals; Biosynthesis; Brain; Brain; C57Bl Mouse; Cell Line, Tumor; Cell Nucleus; Cell Nucleus; Cercopithecus Aethiops; Chlorocebus Aethiops; COS Cell Line; COS Cells; Defects; Diseases; Gene Family; Genetics; Identification; Metabolism; Mice; Mice, Inbred C57BL; Misfolding; Mouse; Mouse; Mutants; Mutation; Mutation; Neuroblastoma; Neuroblastoma; Obesity; Obesity; Obesity; Protein Aggregate; Protein Aggregates; Protein Degradation End Product; Protein Degradation End Products; Protein Folding; Protein Folding; Recessive Mutations; Retinitis Pigmentosa; RNA Splicing; RNA Splicing; Signal Transducing Adaptor Protein; Tub Protein, Mouse; Tubby; TULP1; Tumor Cell Line
- 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.
- The Biochemical Society of the Republic of Korea
- Related Researcher
Oh, Yong Seok
Laboratory of Neural Signaling Networks
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- Brain and Cognitive SciencesETC1. Journal Articles
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