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Target intelligence / Profile preview
Rho-related BTB domain-containing protein 3 (RHOBTB3)
- Target
- RHOBTB3
- Molecular classification
- Atypical Rho GTPase family member, ATPase, Scaffold protein (for E3 ubiquitin ligase complexes), Other (multi-domain regulatory protein with BTB and Rho domains)
01
Overview
Rho-related BTB domain-containing protein 3 (RHOBTB3) is an atypical member of the Rho GTPase superfamily characterized by the presence of a Rho-related domain at its N-terminus and a BTB domain near its C-terminus. Unlike canonical Rho GTPases, RHOBTB3 functions as an ATPase rather than a GTPase and is required for retrograde transport from endosomes to the Golgi apparatus, acting in concert with the Rab9 GTPase to promote vesicle docking and fusion at the Golgi complex. RHOBTB3 serves as a scaffold in a multiprotein E3 ubiquitin ligase complex regulating the turnover of HIFα, thus potentially influencing hypoxia responses. It is expressed in numerous tissues and participates in essential cellular processes involving membrane dynamics and protein trafficking. While there is evidence implicating RHOBTB3 in inherited retinal disease and potentially in neoplasms, there are no approved therapeutics directly targeting this protein at present.
- Other names
- RHOBTB3Rho related BTB domain containing 3KIAA0878Rho-related BTB domain-containing protein 3
02
Biological functions
Retrograde transport (endosome to Golgi)Vesicle docking and fusion at the GolgiScaffold for multi-subunit complexes facilitating HIFα hydroxylation, ubiquitination, and degradationProtein-protein interaction with Rab GTPases, specifically Rab9Regulation of protein localization in endomembrane systems
03
Disease associations
Macular dystrophy, retinal, 3Neoplasms (inferred from genetic/functional studies in mice)Potential indirect role in cancer biology via proteostasis regulation of HIFα (hypoxia-inducible factor alpha)
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