| UniProt ID | CPT1A_HUMAN | |
|---|---|---|
| UniProt AC | P50416 | |
| Protein Name | Carnitine O-palmitoyltransferase 1, liver isoform | |
| Gene Name | CPT1A | |
| Organism | Homo sapiens (Human). | |
| Sequence Length | 773 | |
| Subcellular Localization |
Mitochondrion outer membrane Multi-pass membrane protein . |
|
| Protein Description | Catalyzes the transfer of the acyl group of long-chain fatty acid-CoA conjugates onto carnitine, an essential step for the mitochondrial uptake of long-chain fatty acids and their subsequent beta-oxidation in the mitochondrion. Plays an important role in triglyceride metabolism.. | |
| Protein Sequence | MAEAHQAVAFQFTVTPDGIDLRLSHEALRQIYLSGLHSWKKKFIRFKNGIITGVYPASPSSWLIVVVGVMTTMYAKIDPSLGIIAKINRTLETANCMSSQTKNVVSGVLFGTGLWVALIVTMRYSLKVLLSYHGWMFTEHGKMSRATKIWMGMVKIFSGRKPMLYSFQTSLPRLPVPAVKDTVNRYLQSVRPLMKEEDFKRMTALAQDFAVGLGPRLQWYLKLKSWWATNYVSDWWEEYIYLRGRGPLMVNSNYYAMDLLYILPTHIQAARAGNAIHAILLYRRKLDREEIKPIRLLGSTIPLCSAQWERMFNTSRIPGEETDTIQHMRDSKHIVVYHRGRYFKVWLYHDGRLLKPREMEQQMQRILDNTSEPQPGEARLAALTAGDRVPWARCRQAYFGRGKNKQSLDAVEKAAFFVTLDETEEGYRSEDPDTSMDSYAKSLLHGRCYDRWFDKSFTFVVFKNGKMGLNAEHSWADAPIVAHLWEYVMSIDSLQLGYAEDGHCKGDINPNIPYPTRLQWDIPGECQEVIETSLNTANLLANDVDFHSFPFVAFGKGIIKKCRTSPDAFVQLALQLAHYKDMGKFCLTYEASMTRLFREGRTETVRSCTTESCDFVRAMVDPAQTVEQRLKLFKLASEKHQHMYRLAMTGSGIDRHLFCLYVVSKYLAVESPFLKEVLSEPWRLSTSQTPQQQVELFDLENNPEYVSSGGGFGPVADDGYGVSYILVGENLINFHISSKFSCPETDSHRFGRHLKEAMTDIITLFGLSSNSKK | |
| 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 |
|---|---|---|---|---|---|
| 2 | Acetylation | ------MAEAHQAVA ------CCCCCCEEE | 23.76 | - | |
| 40 | Ubiquitination | LSGLHSWKKKFIRFK HHCHHHHHHHEEEEE | 49.45 | 21890473 | |
| 86 | Ubiquitination | PSLGIIAKINRTLET CCCCHHHHHHCHHHH | 30.06 | 21890473 | |
| 86 (in isoform 2) | Ubiquitination | - | 30.06 | 21890473 | |
| 86 (in isoform 1) | Ubiquitination | - | 30.06 | 21890473 | |
| 86 | 2-Hydroxyisobutyrylation | PSLGIIAKINRTLET CCCCHHHHHHCHHHH | 30.06 | - | |
| 106 | Phosphorylation | SQTKNVVSGVLFGTG CCCCCCCCHHHHCCH | 20.92 | - | |
| 125 | Phosphorylation | LIVTMRYSLKVLLSY HHHHHHHHHHHHHHH | 16.02 | 24719451 | |
| 131 | Phosphorylation | YSLKVLLSYHGWMFT HHHHHHHHHCCCEEE | 15.78 | 29759185 | |
| 132 | Phosphorylation | SLKVLLSYHGWMFTE HHHHHHHHCCCEEEC | 12.34 | 29759185 | |
| 158 | Phosphorylation | MGMVKIFSGRKPMLY HEEHHHHCCCCCEEE | 40.96 | 24719451 | |
| 161 (in isoform 2) | Ubiquitination | - | 38.00 | 21890473 | |
| 161 (in isoform 1) | Ubiquitination | - | 38.00 | 21890473 | |
| 161 | Ubiquitination | VKIFSGRKPMLYSFQ HHHHCCCCCEEEEEC | 38.00 | 21890473 | |
| 165 | Phosphorylation | SGRKPMLYSFQTSLP CCCCCEEEEECCCCC | 10.94 | 27732954 | |
| 166 | Phosphorylation | GRKPMLYSFQTSLPR CCCCEEEEECCCCCC | 13.60 | 27732954 | |
| 169 | Phosphorylation | PMLYSFQTSLPRLPV CEEEEECCCCCCCCC | 30.16 | 27732954 | |
| 170 | Phosphorylation | MLYSFQTSLPRLPVP EEEEECCCCCCCCCC | 26.12 | 27732954 | |
| 180 | Ubiquitination | RLPVPAVKDTVNRYL CCCCCCHHHHHHHHH | 50.19 | 21890473 | |
| 180 | 2-Hydroxyisobutyrylation | RLPVPAVKDTVNRYL CCCCCCHHHHHHHHH | 50.19 | - | |
| 180 | Malonylation | RLPVPAVKDTVNRYL CCCCCCHHHHHHHHH | 50.19 | 26320211 | |
| 180 (in isoform 1) | Ubiquitination | - | 50.19 | 21890473 | |
| 180 (in isoform 2) | Ubiquitination | - | 50.19 | 21890473 | |
| 189 | Phosphorylation | TVNRYLQSVRPLMKE HHHHHHHHHHHHCCH | 19.94 | 24532841 | |
| 195 (in isoform 1) | Ubiquitination | - | 66.68 | 21890473 | |
| 195 (in isoform 2) | Ubiquitination | - | 66.68 | 21890473 | |
| 195 | Ubiquitination | QSVRPLMKEEDFKRM HHHHHHCCHHHHHHH | 66.68 | 21890473 | |
| 200 | Ubiquitination | LMKEEDFKRMTALAQ HCCHHHHHHHHHHHH | 54.29 | - | |
| 282 | Nitration | AIHAILLYRRKLDRE HHHHHHHHHCCCCHH | 12.62 | - | |
| 282 | Nitrated tyrosine | AIHAILLYRRKLDRE HHHHHHHHHCCCCHH | 12.62 | - | |
| 292 | 2-Hydroxyisobutyrylation | KLDREEIKPIRLLGS CCCHHHCCCCEECCC | 37.36 | - | |
| 292 | Ubiquitination | KLDREEIKPIRLLGS CCCHHHCCCCEECCC | 37.36 | - | |
| 322 | Phosphorylation | SRIPGEETDTIQHMR CCCCCCCCCCCCHHH | 33.85 | - | |
| 324 | Phosphorylation | IPGEETDTIQHMRDS CCCCCCCCCCHHHHC | 29.92 | 29759185 | |
| 328 | Sulfoxidation | ETDTIQHMRDSKHIV CCCCCCHHHHCCEEE | 2.67 | 21406390 | |
| 331 | Phosphorylation | TIQHMRDSKHIVVYH CCCHHHHCCEEEEEE | 18.77 | 29759185 | |
| 371 | Phosphorylation | QRILDNTSEPQPGEA HHHHHCCCCCCCCHH | 54.24 | 21712546 | |
| 384 | Phosphorylation | EARLAALTAGDRVPW HHHHHHHHCCCCCCH | 24.70 | 23532336 | |
| 405 (in isoform 2) | Ubiquitination | - | 44.76 | 21890473 | |
| 405 (in isoform 1) | Ubiquitination | - | 44.76 | 21890473 | |
| 405 | Ubiquitination | YFGRGKNKQSLDAVE HCCCCCCHHHHHHHH | 44.76 | 21906983 | |
| 441 | Ubiquitination | TSMDSYAKSLLHGRC CCHHHHHHHHHCCCH | 33.14 | - | |
| 466 | Ubiquitination | FVVFKNGKMGLNAEH EEEEECCEEEECCCC | 38.91 | - | |
| 514 | Phosphorylation | DINPNIPYPTRLQWD CCCCCCCCCCCEECC | 17.28 | 28152594 | |
| 516 | Phosphorylation | NPNIPYPTRLQWDIP CCCCCCCCCEECCCC | 37.86 | 28152594 | |
| 588 | Phosphorylation | DMGKFCLTYEASMTR HHCHHHHEHHHHHHH | 22.09 | - | |
| 589 | Nitration | MGKFCLTYEASMTRL HCHHHHEHHHHHHHH | 9.13 | - | |
| 589 | Nitrated tyrosine | MGKFCLTYEASMTRL HCHHHHEHHHHHHHH | 9.13 | - | |
| 604 | Phosphorylation | FREGRTETVRSCTTE HHCCCCCCHHHCCCC | 22.17 | - | |
| 619 | Sulfoxidation | SCDFVRAMVDPAQTV CCHHHHHHCCHHHHH | 2.17 | 21406390 | |
| 634 | Acetylation | EQRLKLFKLASEKHQ HHHHHHHHHHHHHHH | 54.29 | 25953088 | |
| 634 | Ubiquitination | EQRLKLFKLASEKHQ HHHHHHHHHHHHHHH | 54.29 | - | |
| 648 | Sulfoxidation | QHMYRLAMTGSGIDR HHHHHHHHCCCCHHH | 5.30 | 21406390 | |
| 649 | Phosphorylation | HMYRLAMTGSGIDRH HHHHHHHCCCCHHHH | 23.69 | 20860994 | |
| 651 | Phosphorylation | YRLAMTGSGIDRHLF HHHHHCCCCHHHHHH | 24.33 | 20860994 | |
| 666 | Phosphorylation | CLYVVSKYLAVESPF HHHHHHHHHHHCCHH | 7.74 | 21406692 | |
| 671 | Phosphorylation | SKYLAVESPFLKEVL HHHHHHCCHHHHHHH | 17.85 | 24719451 | |
| 675 (in isoform 1) | Ubiquitination | - | 46.27 | 21890473 | |
| 675 (in isoform 2) | Ubiquitination | - | 46.27 | 21890473 | |
| 675 | Ubiquitination | AVESPFLKEVLSEPW HHCCHHHHHHHCCCC | 46.27 | 2190698 | |
| 741 | Phosphorylation | FHISSKFSCPETDSH EEECCCCCCCCCCCH | 32.28 | 21082442 | |
| 747 | Phosphorylation | FSCPETDSHRFGRHL CCCCCCCCHHHHHHH | 24.79 | 21082442 | |
| 755 | Ubiquitination | HRFGRHLKEAMTDII HHHHHHHHHHHHHHH | 36.98 | - | |
| 768 | Phosphorylation | IITLFGLSSNSKK-- HHHHHCCCCCCCC-- | 28.05 | 28348404 | |
| 769 | Phosphorylation | ITLFGLSSNSKK--- HHHHCCCCCCCC--- | 50.60 | 28348404 | |
| 771 | Phosphorylation | LFGLSSNSKK----- HHCCCCCCCC----- | 43.71 | 29457462 | |
| 772 (in isoform 1) | Ubiquitination | - | 67.75 | 21890473 | |
| 772 | Ubiquitination | FGLSSNSKK------ HCCCCCCCC------ | 67.75 | - | |
| 773 | Ubiquitination | GLSSNSKK------- CCCCCCCC------- | 66.45 | - |
| 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 CPT1A_HUMAN !! | ||||||
| Modified Location | Modified Residue | Modification | Function | Reference | ||
|---|---|---|---|---|---|---|
Oops, there are no descriptions of PTM sites of CPT1A_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 CPT1A_HUMAN !! | ||||||
| Interacting Protein | Gene Name | Interaction Type | PPI Reference | Domain-Domain Interactions |
|---|---|---|---|---|
| EIF3C_HUMAN | EIF3C | physical | 22939629 | |
| LPP_HUMAN | LPP | physical | 22939629 | |
| TOM40_HUMAN | TOMM40 | physical | 22939629 | |
| VATC1_HUMAN | ATP6V1C1 | physical | 22939629 | |
| RHOA_HUMAN | RHOA | physical | 22939629 | |
| NDUA2_HUMAN | NDUFA2 | physical | 22939629 | |
| ERLN1_HUMAN | ERLIN1 | physical | 22939629 | |
| NDUV1_HUMAN | NDUFV1 | physical | 22939629 | |
| NDUS1_HUMAN | NDUFS1 | physical | 22939629 | |
| PCBP1_HUMAN | PCBP1 | physical | 22939629 | |
| U520_HUMAN | SNRNP200 | physical | 22939629 | |
| NPDC1_HUMAN | NPDC1 | physical | 21988832 | |
| CY24A_HUMAN | CYBA | physical | 21988832 | |
| NR4A1_HUMAN | NR4A1 | physical | 21988832 | |
| GRP78_HUMAN | HSPA5 | physical | 21988832 | |
| KBTB7_HUMAN | KBTBD7 | physical | 21988832 |
| Kegg Disease | |
|---|---|
| H00525 | Disorders of fatty-acid oxidation, including: Medium-chain (MC) acyl-CoA dehydrogenase (AD) deficien |
| OMIM Disease | |
| 255120 | Carnitine palmitoyltransferase 1A deficiency (CPT1AD) |
| Kegg Drug | |
| D05292 | Oxfenicine (USAN/INN) |
| D05442 | Perhexiline maleate (USAN) |
| D08340 | Perhexiline (INN) |
| DrugBank | |
| DB01016 | Glyburide |
| DB00583 | L-Carnitine |
| DB01074 | Perhexiline |
loading...