UniProt ID | ATP7A_HUMAN | |
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UniProt AC | Q04656 | |
Protein Name | Copper-transporting ATPase 1 | |
Gene Name | ATP7A | |
Organism | Homo sapiens (Human). | |
Sequence Length | 1500 | |
Subcellular Localization |
Golgi apparatus, trans-Golgi network membrane Multi-pass membrane protein . Cell membrane Multi-pass membrane protein . Cycles constitutively between the trans-Golgi network (TGN) and the plasma membrane (PubMed:9147644). Predominantly found in t |
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Protein Description | May supply copper to copper-requiring proteins within the secretory pathway, when localized in the trans-Golgi network. Under conditions of elevated extracellular copper, it relocalized to the plasma membrane where it functions in the efflux of copper from cells.. | |
Protein Sequence | MDPSMGVNSVTISVEGMTCNSCVWTIEQQIGKVNGVHHIKVSLEEKNATIIYDPKLQTPKTLQEAIDDMGFDAVIHNPDPLPVLTDTLFLTVTASLTLPWDHIQSTLLKTKGVTDIKIYPQKRTVAVTIIPSIVNANQIKELVPELSLDTGTLEKKSGACEDHSMAQAGEVVLKMKVEGMTCHSCTSTIEGKIGKLQGVQRIKVSLDNQEATIVYQPHLISVEEMKKQIEAMGFPAFVKKQPKYLKLGAIDVERLKNTPVKSSEGSQQRSPSYTNDSTATFIIDGMHCKSCVSNIESTLSALQYVSSIVVSLENRSAIVKYNASSVTPESLRKAIEAVSPGLYRVSITSEVESTSNSPSSSSLQKIPLNVVSQPLTQETVINIDGMTCNSCVQSIEGVISKKPGVKSIRVSLANSNGTVEYDPLLTSPETLRGAIEDMGFDATLSDTNEPLVVIAQPSSEMPLLTSTNEFYTKGMTPVQDKEEGKNSSKCYIQVTGMTCASCVANIERNLRREEGIYSILVALMAGKAEVRYNPAVIQPPMIAEFIRELGFGATVIENADEGDGVLELVVRGMTCASCVHKIESSLTKHRGILYCSVALATNKAHIKYDPEIIGPRDIIHTIESLGFEASLVKKDRSASHLDHKREIRQWRRSFLVSLFFCIPVMGLMIYMMVMDHHFATLHHNQNMSKEEMINLHSSMFLERQILPGLSVMNLLSFLLCVPVQFFGGWYFYIQAYKALKHKTANMDVLIVLATTIAFAYSLIILLVAMYERAKVNPITFFDTPPMLFVFIALGRWLEHIAKGKTSEALAKLISLQATEATIVTLDSDNILLSEEQVDVELVQRGDIIKVVPGGKFPVDGRVIEGHSMVDESLITGEAMPVAKKPGSTVIAGSINQNGSLLICATHVGADTTLSQIVKLVEEAQTSKAPIQQFADKLSGYFVPFIVFVSIATLLVWIVIGFLNFEIVETYFPGYNRSISRTETIIRFAFQASITVLCIACPCSLGLATPTAVMVGTGVGAQNGILIKGGEPLEMAHKVKVVVFDKTGTITHGTPVVNQVKVLTESNRISHHKILAIVGTAESNSEHPLGTAITKYCKQELDTETLGTCIDFQVVPGCGISCKVTNIEGLLHKNNWNIEDNNIKNASLVQIDASNEQSSTSSSMIIDAQISNALNAQQYKVLIGNREWMIRNGLVINNDVNDFMTEHERKGRTAVLVAVDDELCGLIAIADTVKPEAELAIHILKSMGLEVVLMTGDNSKTARSIASQVGITKVFAEVLPSHKVAKVKQLQEEGKRVAMVGDGINDSPALAMANVGIAIGTGTDVAIEAADVVLIRNDLLDVVASIDLSRETVKRIRINFVFALIYNLVGIPIAAGVFMPIGLVLQPWMGSAAMAASSVSVVLSSLFLKLYRKPTYESYELPARSQIGQKSPSEISVHVGIDDTSRNSPKLGLLDRIVNYSRASINSLLSDKRSLNSVVTSEPDKHSLLVGDFREDDDTAL | |
Overview of Protein Modification Sites with Functional and Structural Information | ||
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* ASA = Accessible Surface Area
Locations | Modification | Substrate Peptides & Secondary Structure |
ASA (%) | Reference | Orthologous Protein Cluster |
---|---|---|---|---|---|
58 | Phosphorylation | IYDPKLQTPKTLQEA EECCCCCCCHHHHHH | 36.87 | 24719451 | |
147 | Phosphorylation | KELVPELSLDTGTLE HHHHCCCCCCCCCCE | 23.65 | 29255136 | |
150 | Phosphorylation | VPELSLDTGTLEKKS HCCCCCCCCCCEECC | 37.04 | 30266825 | |
152 | Phosphorylation | ELSLDTGTLEKKSGA CCCCCCCCCEECCCC | 33.56 | 30266825 | |
155 | Ubiquitination | LDTGTLEKKSGACED CCCCCCEECCCCCCC | 56.18 | - | |
195 | Ubiquitination | TIEGKIGKLQGVQRI EECCCCCEECCEEEE | 40.90 | - | |
226 | Acetylation | LISVEEMKKQIEAMG ECCHHHHHHHHHHCC | 44.82 | 7469333 | |
227 | Acetylation | ISVEEMKKQIEAMGF CCHHHHHHHHHHCCC | 56.02 | 7469345 | |
239 | Acetylation | MGFPAFVKKQPKYLK CCCCHHHCCCCCEEE | 38.96 | 7469357 | |
244 | Phosphorylation | FVKKQPKYLKLGAID HHCCCCCEEECCCCC | 18.76 | - | |
246 | Ubiquitination | KKQPKYLKLGAIDVE CCCCCEEECCCCCHH | 41.61 | - | |
262 | Phosphorylation | LKNTPVKSSEGSQQR HCCCCCCCCCCCCCC | 33.58 | 30108239 | |
263 | Phosphorylation | KNTPVKSSEGSQQRS CCCCCCCCCCCCCCC | 40.01 | 30108239 | |
266 | Phosphorylation | PVKSSEGSQQRSPSY CCCCCCCCCCCCCCC | 21.06 | 30108239 | |
270 | Phosphorylation | SEGSQQRSPSYTNDS CCCCCCCCCCCCCCC | 17.54 | 25159151 | |
272 | Phosphorylation | GSQQRSPSYTNDSTA CCCCCCCCCCCCCCE | 45.92 | 27794612 | |
273 | Phosphorylation | SQQRSPSYTNDSTAT CCCCCCCCCCCCCEE | 16.97 | 21712546 | |
274 | Phosphorylation | QQRSPSYTNDSTATF CCCCCCCCCCCCEEE | 36.57 | 21712546 | |
277 | Phosphorylation | SPSYTNDSTATFIID CCCCCCCCCEEEEEE | 23.24 | 23663014 | |
278 | Phosphorylation | PSYTNDSTATFIIDG CCCCCCCCEEEEEEC | 32.36 | 23898821 | |
280 | Phosphorylation | YTNDSTATFIIDGMH CCCCCCEEEEEECCC | 18.56 | 23663014 | |
316 | Phosphorylation | VVSLENRSAIVKYNA EEEECCCCEEEEEEC | 33.65 | 29759185 | |
321 | Phosphorylation | NRSAIVKYNASSVTP CCCEEEEEECCCCCH | 12.69 | 29759185 | |
324 | Phosphorylation | AIVKYNASSVTPESL EEEEEECCCCCHHHH | 23.00 | 28450419 | |
325 | Phosphorylation | IVKYNASSVTPESLR EEEEECCCCCHHHHH | 27.83 | 28450419 | |
327 | Phosphorylation | KYNASSVTPESLRKA EEECCCCCHHHHHHH | 24.24 | 25159151 | |
330 | Phosphorylation | ASSVTPESLRKAIEA CCCCCHHHHHHHHHH | 34.38 | 28450419 | |
333 | Ubiquitination | VTPESLRKAIEAVSP CCHHHHHHHHHHHCC | 60.04 | 22053931 | |
333 (in isoform 1) | Ubiquitination | - | 60.04 | 22053931 | |
339 | Phosphorylation | RKAIEAVSPGLYRVS HHHHHHHCCCEEEEE | 21.94 | 25159151 | |
343 | Phosphorylation | EAVSPGLYRVSITSE HHHCCCEEEEEEEEE | 18.19 | 20068231 | |
346 | Phosphorylation | SPGLYRVSITSEVES CCCEEEEEEEEEEEE | 15.91 | 23403867 | |
348 | Phosphorylation | GLYRVSITSEVESTS CEEEEEEEEEEEECC | 16.01 | 23663014 | |
349 | Phosphorylation | LYRVSITSEVESTSN EEEEEEEEEEEECCC | 37.67 | 30177828 | |
353 | Phosphorylation | SITSEVESTSNSPSS EEEEEEEECCCCCCC | 42.16 | 30266825 | |
354 | Phosphorylation | ITSEVESTSNSPSSS EEEEEEECCCCCCCC | 20.58 | 30266825 | |
355 | Phosphorylation | TSEVESTSNSPSSSS EEEEEECCCCCCCCC | 44.59 | 30266825 | |
357 | Phosphorylation | EVESTSNSPSSSSLQ EEEECCCCCCCCCCC | 26.40 | 30266825 | |
359 | Phosphorylation | ESTSNSPSSSSLQKI EECCCCCCCCCCCCC | 42.55 | 30266825 | |
360 | Phosphorylation | STSNSPSSSSLQKIP ECCCCCCCCCCCCCC | 27.59 | 30266825 | |
361 | Phosphorylation | TSNSPSSSSLQKIPL CCCCCCCCCCCCCCC | 39.13 | 30266825 | |
362 | Phosphorylation | SNSPSSSSLQKIPLN CCCCCCCCCCCCCCC | 36.45 | 29255136 | |
372 | Phosphorylation | KIPLNVVSQPLTQET CCCCCEECCCCCCCE | 23.07 | 26074081 | |
376 | Phosphorylation | NVVSQPLTQETVINI CEECCCCCCCEEEEE | 30.92 | 26074081 | |
426 | Phosphorylation | VEYDPLLTSPETLRG EEECCCCCCHHHHHH | 50.33 | 25002506 | |
427 | Phosphorylation | EYDPLLTSPETLRGA EECCCCCCHHHHHHH | 22.25 | 25159151 | |
430 | Phosphorylation | PLLTSPETLRGAIED CCCCCHHHHHHHHHH | 25.74 | 24719451 | |
445 | Phosphorylation | MGFDATLSDTNEPLV HCCCEECCCCCCCEE | 37.73 | 30576142 | |
447 | Phosphorylation | FDATLSDTNEPLVVI CCEECCCCCCCEEEE | 37.80 | 30576142 | |
465 | Phosphorylation | SSEMPLLTSTNEFYT CCCCCCCEECCCEEC | 40.98 | 30576142 | |
467 | Phosphorylation | EMPLLTSTNEFYTKG CCCCCEECCCEECCC | 33.27 | 30576142 | |
471 | Phosphorylation | LTSTNEFYTKGMTPV CEECCCEECCCCCCC | 11.02 | 25884760 | |
476 | Phosphorylation | EFYTKGMTPVQDKEE CEECCCCCCCCCHHC | 29.04 | 29255136 | |
517 | Phosphorylation | LRREEGIYSILVALM HHHHCCHHHHHHHHH | 10.66 | 18180459 | |
518 | Phosphorylation | RREEGIYSILVALMA HHHCCHHHHHHHHHC | 13.92 | - | |
594 | Phosphorylation | TKHRGILYCSVALAT HHCCCCHHEEEEECC | 4.83 | - | |
601 | Phosphorylation | YCSVALATNKAHIKY HEEEEECCCCCCCCC | 37.79 | - | |
630 | Phosphorylation | ESLGFEASLVKKDRS HHCCCEEEEEECCCC | 26.88 | 24719451 | |
633 | Ubiquitination | GFEASLVKKDRSASH CCEEEEEECCCCCCC | 55.00 | - | |
639 | Phosphorylation | VKKDRSASHLDHKRE EECCCCCCCCCHHHH | 26.32 | 28348404 | |
686 | N-linked_Glycosylation | ATLHHNQNMSKEEMI HHHCCCCCCCHHHHH | 41.85 | UniProtKB CARBOHYD | |
743 | Phosphorylation | YKALKHKTANMDVLI HHHHCCCCCCCHHHH | 24.00 | 25262027 | |
754 | Phosphorylation | DVLIVLATTIAFAYS HHHHHHHHHHHHHHH | 17.99 | 25262027 | |
755 | Phosphorylation | VLIVLATTIAFAYSL HHHHHHHHHHHHHHH | 12.52 | 25262027 | |
760 | Phosphorylation | ATTIAFAYSLIILLV HHHHHHHHHHHHHHH | 9.50 | 25262027 | |
761 | Phosphorylation | TTIAFAYSLIILLVA HHHHHHHHHHHHHHH | 15.06 | 25262027 | |
770 | Phosphorylation | IILLVAMYERAKVNP HHHHHHHHHHHCCCC | 7.79 | 25262027 | |
827 | Phosphorylation | ATIVTLDSDNILLSE EEEEEECCCCEEECH | 35.35 | - | |
849 | Ubiquitination | VQRGDIIKVVPGGKF EECCCEEEECCCCCC | 37.20 | - | |
855 | Ubiquitination | IKVVPGGKFPVDGRV EEECCCCCCCCCCEE | 53.03 | - | |
936 (in isoform 3) | Ubiquitination | - | 40.91 | - | |
975 | N-linked_Glycosylation | ETYFPGYNRSISRTE HHHCCCCCCCCCCHH | 36.82 | UniProtKB CARBOHYD | |
1037 | Ubiquitination | EPLEMAHKVKVVVFD CCCCCCEEEEEEEEC | 33.92 | - | |
1039 | Ubiquitination | LEMAHKVKVVVFDKT CCCCEEEEEEEECCC | 33.83 | - | |
1045 | Ubiquitination | VKVVVFDKTGTITHG EEEEEECCCCCCCCC | 37.07 | - | |
1046 | Phosphorylation | KVVVFDKTGTITHGT EEEEECCCCCCCCCC | 41.21 | 23312004 | |
1048 | Phosphorylation | VVFDKTGTITHGTPV EEECCCCCCCCCCCE | 28.12 | 23312004 | |
1050 | Phosphorylation | FDKTGTITHGTPVVN ECCCCCCCCCCCEEC | 17.68 | 23312004 | |
1053 | Phosphorylation | TGTITHGTPVVNQVK CCCCCCCCCEECEEE | 12.81 | 23312004 | |
1060 | Ubiquitination | TPVVNQVKVLTESNR CCEECEEEEEECCCC | 23.05 | - | |
1132 | Ubiquitination | NIEGLLHKNNWNIED CHHHHCCCCCCCCCC | 52.53 | - | |
1146 | Phosphorylation | DNNIKNASLVQIDAS CCCCCCCEEEEEECC | 38.17 | 26074081 | |
1157 | Phosphorylation | IDASNEQSSTSSSMI EECCCCCCCCCCCEE | 29.42 | 22210691 | |
1158 | Phosphorylation | DASNEQSSTSSSMII ECCCCCCCCCCCEEH | 31.81 | 22210691 | |
1178 | Phosphorylation | NALNAQQYKVLIGNR HCCCHHHEEEEECCC | 7.32 | 22210691 | |
1204 | Phosphorylation | NDVNDFMTEHERKGR CCHHHHHHHHHHCCC | 33.90 | - | |
1212 | Phosphorylation | EHERKGRTAVLVAVD HHHHCCCEEEEEEEC | 29.77 | - | |
1245 | Phosphorylation | LAIHILKSMGLEVVL HHHHHHHHCCCEEEE | 18.03 | 28270605 | |
1254 | Phosphorylation | GLEVVLMTGDNSKTA CCEEEEECCCCHHHH | 36.90 | 28270605 | |
1258 | Phosphorylation | VLMTGDNSKTARSIA EEECCCCHHHHHHHH | 35.80 | 28270605 | |
1306 | Phosphorylation | VGDGINDSPALAMAN ECCCCCCCHHHHHCC | 13.95 | - | |
1410 | Phosphorylation | SSLFLKLYRKPTYES HHHHHHHHCCCCCCC | 18.27 | 26074081 | |
1412 | Ubiquitination | LFLKLYRKPTYESYE HHHHHHCCCCCCCCC | 28.31 | - | |
1414 | Phosphorylation | LKLYRKPTYESYELP HHHHCCCCCCCCCCC | 42.85 | 26074081 | |
1415 | Phosphorylation | KLYRKPTYESYELPA HHHCCCCCCCCCCCC | 16.05 | 26074081 | |
1417 | Phosphorylation | YRKPTYESYELPARS HCCCCCCCCCCCCHH | 17.31 | 26074081 | |
1418 | Phosphorylation | RKPTYESYELPARSQ CCCCCCCCCCCCHHH | 15.00 | 26074081 | |
1424 | Phosphorylation | SYELPARSQIGQKSP CCCCCCHHHCCCCCC | 28.40 | - | |
1430 | Phosphorylation | RSQIGQKSPSEISVH HHHCCCCCCCCEEEE | 26.27 | 25159151 | |
1432 | Phosphorylation | QIGQKSPSEISVHVG HCCCCCCCCEEEEEE | 55.44 | 21955146 | |
1435 | Phosphorylation | QKSPSEISVHVGIDD CCCCCCEEEEEECCC | 11.34 | 25219547 | |
1443 | Phosphorylation | VHVGIDDTSRNSPKL EEEECCCCCCCCCCC | 26.32 | 25159151 | |
1444 | Phosphorylation | HVGIDDTSRNSPKLG EEECCCCCCCCCCCC | 36.14 | 25159151 | |
1447 | Phosphorylation | IDDTSRNSPKLGLLD CCCCCCCCCCCCHHH | 23.46 | 25159151 | |
1449 | Ubiquitination | DTSRNSPKLGLLDRI CCCCCCCCCCHHHHH | 55.82 | - | |
1459 | Phosphorylation | LLDRIVNYSRASINS HHHHHHHCCHHHHHH | 6.66 | 25884760 | |
1460 | Phosphorylation | LDRIVNYSRASINSL HHHHHHCCHHHHHHH | 19.03 | 30108239 | |
1463 | Phosphorylation | IVNYSRASINSLLSD HHHCCHHHHHHHHCC | 22.61 | 23401153 | |
1466 | Phosphorylation | YSRASINSLLSDKRS CCHHHHHHHHCCCCC | 29.08 | 23401153 | |
1469 | Phosphorylation | ASINSLLSDKRSLNS HHHHHHHCCCCCCCC | 46.51 | 30266825 | |
1471 | Ubiquitination | INSLLSDKRSLNSVV HHHHHCCCCCCCCCC | 40.28 | - | |
1473 | Phosphorylation | SLLSDKRSLNSVVTS HHHCCCCCCCCCCCC | 37.33 | 29255136 | |
1476 | Phosphorylation | SDKRSLNSVVTSEPD CCCCCCCCCCCCCCC | 24.21 | 29255136 | |
1479 | Phosphorylation | RSLNSVVTSEPDKHS CCCCCCCCCCCCCCC | 25.80 | 30108239 | |
1480 | Phosphorylation | SLNSVVTSEPDKHSL CCCCCCCCCCCCCCE | 35.21 | 21815630 | |
1486 | Phosphorylation | TSEPDKHSLLVGDFR CCCCCCCCEEEECCC | 29.19 | 30108239 | |
1498 | Phosphorylation | DFREDDDTAL----- CCCCCCCCCC----- | 36.53 | 24719451 | |
1552 (in isoform 3) | Ubiquitination | - | - |
Modified Location | Modified Residue | Modification | Function | Reference | ||
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Oops, there are no descriptions of PTM sites of ATP7A_HUMAN !! |
* Distance = the distance between SAP position and PTM sites.
Modified Location | Modification | Variant Position (Distance <= 10) |
Residue Change | SAP | Related Disease | Reference |
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Oops, there are no SNP-PTM records of ATP7A_HUMAN !! |
Interacting Protein | Gene Name | Interaction Type | PPI Reference | Domain-Domain Interactions |
---|---|---|---|---|
GLRX1_HUMAN | GLRX | physical | 16884690 | |
ATOX1_HUMAN | ATOX1 | physical | 10497213 | |
CLUS_HUMAN | CLU | physical | 21242307 | |
COMD1_HUMAN | COMMD1 | physical | 22130675 |
Kegg Disease | |
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H00209 | Menkes disease (MD); Menkes kinky hair syndrome |
H00455 | Spinal muscular atrophy (SMA), including: SMA type I (SMA1) / Werdning-Hoffman disease; SMA type II |
H00557 | Cutis laxa, including: Autosomal dominant cutis laxa (ADCL); Autosomal recessive cutis laxa I (ARCL1 |
H00856 | Distal hereditary motor neuropathies (dHMN) |
OMIM Disease | |
309400 | Menkes disease (MNKD) |
304150 | Occipital horn syndrome (OHS) |
300489 | Distal spinal muscular atrophy, X-linked, 3 (DSMAX3) |
Kegg Drug | |
There are no disease associations of PTM sites. | |
DrugBank | |
DB00958 | Carboplatin |
DB00515 | Cisplatin |
DB00526 | Oxaliplatin |
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