| Gene Symbol |
USP30
|
| Entrez Gene |
84749
|
| Alt Symbol |
-
|
| Species |
Human
|
| Gene Type |
protein-coding
|
| Description |
ubiquitin specific peptidase 30
|
| Other Description |
deubiquitinating enzyme 30|ub-specific protease 30|ubiquitin carboxyl-terminal hydrolase 30|ubiquitin specific protease 30|ubiquitin thioesterase 30|ubiquitin thiolesterase 30|ubiquitin-specific protease 30|ubiquitin-specific-processing protease 30
|
| Swissprots |
Q8WTU7 Q9BSS3 Q70CQ3 Q96JX4
|
| Accessions |
CAE89969 EAW97842 EAW97843 EAW97844 Q70CQ3 AA856774 AI867665 AJ586136 CAE51936 AK027820 BAB55392 AK097830 BAG53537 AL834278 BC004868 AAH04868 BC022094 AAH22094 BG149730 BX095894 CN367077 DQ893401 ABM84327 DQ896783 ABM87782 HY127211 XM_005253962 XP_005254019 XM_005253963 XP_005254020 XM_005253965 XP_005254022 XM_006719653 XP_006719716 XM_011538894 XP_011537196 NM_001301175 NP_001288104 NM_032663 NP_116052
|
| Function |
Deubiquitinating enzyme tethered to the mitochondrial outer membrane that acts as a key inhibitor of mitophagy by counteracting the action of parkin (PARK2): hydrolyzes ubiquitin attached by parkin on target proteins, such as RHOT1/MIRO1 and TOMM20, thereby blocking parkin's ability to drive mitophagy (PubMed:18287522, PubMed:24896179, PubMed:25527291, PubMed:25621951). Preferentially cleaves 'Lys-6'- and 'Lys-11'- linked polyubiquitin chains, 2 types of linkage that participate to mitophagic signaling (PubMed:25621951). Does not cleave efficiently polyubiquitin phosphorylated at 'Ser-65' (PubMed:25527291). Acts as negative regulator of mitochondrial fusion by mediating deubiquitination of MFN1 and MFN2 (By similarity). {ECO:0000250|UniProtKB:Q3UN04, ECO:0000269|PubMed:18287522, ECO:0000269|PubMed:24896179, ECO:0000269|PubMed:25527291, ECO:0000269|PubMed:25621951}.
|
| Subcellular Location |
Mitochondrion outer membrane {ECO:0000269|PubMed:18287522, ECO:0000269|PubMed:24896179}.
|
| Tissue Specificity |
Expressed in skeletal muscle, pancreas, liver and kidney. {ECO:0000269|PubMed:14715245}.
|