YOD1
Description
The YOD1 (YOD1 deubiquitinase) is a protein-coding gene located on chromosome 1.
YOD1 is a hydrolase that removes ubiquitin from proteins, playing a crucial role in endoplasmic reticulum-associated degradation (ERAD) of misfolded proteins. It facilitates the threading of these proteins through the VCP/p97 pore by trimming the ubiquitin chain, removing a steric impediment. YOD1 specifically targets 'Lys-27'-, 'Lys-29'-, and 'Lys-33'-linked polyubiquitin chains, and can also hydrolyze 'Lys-11'-linked chains. Additionally, YOD1 cleaves both polyubiquitin and di-ubiquitin. YOD1 is involved in macroautophagy, particularly in the clearance of damaged lysosomes. It recruits PLAA, UBXN6, and VCP to these damaged lysosomes, removing K48-linked ubiquitin chains, which allows for autophagosome formation.
YOD1 is also known as DUBA8, OTUD2, PRO0907.
Associated Diseases
- ovarian cancer
- endometrial cancer
- urinary bladder cancer
- isolated asymptomatic elevation of creatine phosphokinase
- pentosuria
- 22q11.2 deletion syndrome
