| UniProt ID | COQ6_HUMAN | |
|---|---|---|
| UniProt AC | Q9Y2Z9 | |
| Protein Name | Ubiquinone biosynthesis monooxygenase COQ6, mitochondrial {ECO:0000255|HAMAP-Rule:MF_03193} | |
| Gene Name | COQ6 {ECO:0000255|HAMAP-Rule:MF_03193} | |
| Organism | Homo sapiens (Human). | |
| Sequence Length | 468 | |
| Subcellular Localization |
Mitochondrion inner membrane Peripheral membrane protein Matrix side . Golgi apparatus . Cell projection . Localizes to cell processes and Golgi apparatus in podocytes. |
|
| Protein Description | FAD-dependent monooxygenase required for the C5-ring hydroxylation during ubiquinone biosynthesis. Catalyzes the hydroxylation of 3-polyprenyl-4-hydroxybenzoic acid to 3-polyprenyl-4,5-dihydroxybenzoic acid. The electrons required for the hydroxylation reaction may be funneled indirectly from NADPH via a ferredoxin/ferredoxin reductase system to COQ6.. | |
| Protein Sequence | MAARLVSRCGAVRAAPHSGPLVSWRRWSGASTDTVYDVVVSGGGLVGAAMACALGYDIHFHDKKILLLEAGPKKVLEKLSETYSNRVSSISPGSATLLSSFGAWDHICNMRYRAFRRMQVWDACSEALIMFDKDNLDDMGYIVENDVIMHALTKQLEAVSDRVTVLYRSKAIRYTWPCPFPMADSSPWVHITLGDGSTFQTKLLIGADGHNSGVRQAVGIQNVSWNYDQSAVVATLHLSEATENNVAWQRFLPSGPIALLPLSDTLSSLVWSTSHEHAAELVSMDEEKFVDAVNSAFWSDADHTDFIDTAGAMLQYAVSLLKPTKVSARQLPPSVARVDAKSRVLFPLGLGHAAEYVRPRVALIGDAAHRVHPLAGQGVNMGFGDISSLAHHLSTAAFNGKDLGSVSHLTGYETERQRHNTALLAATDLLKRLYSTSASPLVLLRTWGLQATNAVSPLKEQIMAFASK | |
| Overview of Protein Modification Sites with Functional and Structural Information | ||
|
|
||
* ASA = Accessible Surface Area
| Locations | Modification | Substrate Peptides & Secondary Structure |
ASA (%) | Reference | Orthologous Protein Cluster |
|---|---|---|---|---|---|
| 9 (in isoform 3) | Phosphorylation | - | 2.98 | 24245541 | |
| 53 | Ubiquitination | VGAAMACALGYDIHF HHHHHHHHHCCEEEE | 8.42 | 21890473 | |
| 74 | Acetylation | LLEAGPKKVLEKLSE EEECCHHHHHHHHHH | 56.01 | 30587849 | |
| 78 | Ubiquitination | GPKKVLEKLSETYSN CHHHHHHHHHHHHHC | 53.83 | 21890473 | |
| 88 | Phosphorylation | ETYSNRVSSISPGSA HHHHCCCCCCCCCCC | 20.79 | - | |
| 319 | Phosphorylation | AMLQYAVSLLKPTKV HHHHHHHHHHCCCCC | 21.58 | 24719451 | |
| 327 | Phosphorylation | LLKPTKVSARQLPPS HHCCCCCCHHHCCCC | 21.20 | - | |
| 334 | Phosphorylation | SARQLPPSVARVDAK CHHHCCCCCCCCCCC | 26.86 | - | |
| 414 | Phosphorylation | SHLTGYETERQRHNT HHHCCCHHHHHHHHH | 28.16 | - | |
| 421 | O-linked_Glycosylation | TERQRHNTALLAATD HHHHHHHHHHHHHHH | 16.74 | 31492838 | |
| 431 | 2-Hydroxyisobutyrylation | LAATDLLKRLYSTSA HHHHHHHHHHHCCCC | 47.88 | - | |
| 434 | Phosphorylation | TDLLKRLYSTSASPL HHHHHHHHCCCCCHH | 17.59 | 20068231 | |
| 435 | Phosphorylation | DLLKRLYSTSASPLV HHHHHHHCCCCCHHH | 22.07 | 20068231 | |
| 436 | Phosphorylation | LLKRLYSTSASPLVL HHHHHHCCCCCHHHH | 18.11 | 20068231 | |
| 437 | Phosphorylation | LKRLYSTSASPLVLL HHHHHCCCCCHHHHH | 22.32 | 20068231 | |
| 439 | Phosphorylation | RLYSTSASPLVLLRT HHHCCCCCHHHHHHH | 20.66 | 20068231 | |
| 456 | Phosphorylation | LQATNAVSPLKEQIM CCCCCCCCCHHHHHH | 23.31 | 24719451 | |
| 459 | Ubiquitination | TNAVSPLKEQIMAFA CCCCCCHHHHHHHHH | 51.46 | - |
| Modified Location | Modified Residue | Modification | Type of Upstream Proteins | Gene Name of Upstream Proteins | UniProt AC of Upstream Proteins | Sources |
|---|---|---|---|---|---|---|
Oops, there are no upstream regulatory protein records of COQ6_HUMAN !! | ||||||
| Modified Location | Modified Residue | Modification | Function | Reference | ||
|---|---|---|---|---|---|---|
Oops, there are no descriptions of PTM sites of COQ6_HUMAN !! | ||||||
* Distance = the distance between SAP position and PTM sites.
| Modified Location | Modification | Variant Position (Distance <= 10) |
Residue Change | SAP | Related Disease | Reference |
|---|---|---|---|---|---|---|
Oops, there are no SNP-PTM records of COQ6_HUMAN !! | ||||||
| Interacting Protein | Gene Name | Interaction Type | PPI Reference | Domain-Domain Interactions |
|---|---|---|---|---|
| ZBT16_HUMAN | ZBTB16 | physical | 16169070 | |
| A4_HUMAN | APP | physical | 21832049 | |
| COQ3_HUMAN | COQ3 | physical | 26344197 | |
| COQ5_HUMAN | COQ5 | physical | 27499296 | |
| COQ6_HUMAN | COQ6 | physical | 27499296 | |
| NDUF5_HUMAN | NDUFAF5 | physical | 27499296 | |
| DCMC_HUMAN | MLYCD | physical | 27499296 | |
| BOLA3_HUMAN | BOLA3 | physical | 27499296 | |
| NRDC_HUMAN | NRD1 | physical | 27499296 | |
| MPPB_HUMAN | PMPCB | physical | 27499296 | |
| MPPA_HUMAN | PMPCA | physical | 27499296 | |
| CHCH2_HUMAN | CHCHD2 | physical | 27499296 | |
| PDIP2_HUMAN | POLDIP2 | physical | 27499296 | |
| ECH1_HUMAN | ECH1 | physical | 27499296 | |
| CYC_HUMAN | CYCS | physical | 27499296 | |
| ATP5E_HUMAN | ATP5E | physical | 27499296 | |
| ATPG_HUMAN | ATP5C1 | physical | 27499296 | |
| C1QBP_HUMAN | C1QBP | physical | 27499296 | |
| NIT2_HUMAN | NIT2 | physical | 27499296 | |
| RM13_HUMAN | MRPL13 | physical | 27499296 | |
| ODBA_HUMAN | BCKDHA | physical | 27499296 | |
| ATP5J_HUMAN | ATP5J | physical | 27499296 | |
| ETFA_HUMAN | ETFA | physical | 27499296 |
| Kegg Disease | ||||||
|---|---|---|---|---|---|---|
| There are no disease associations of PTM sites. | ||||||
| OMIM Disease | ||||||
| 614650 | Coenzyme Q10 deficiency, primary, 6 (COQ10D6) | |||||
| Kegg Drug | ||||||
| There are no disease associations of PTM sites. | ||||||
| DrugBank | ||||||
| There are no disease associations of PTM sites. | ||||||
loading...