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. |
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