Protein:ADAMTS13 |
Protein Summary |
 Gene summary |
| Gene name: ADAMTS13 | ASpdb.0 ID: 11093 | Gene | Gene symbol | ADAMTS13 | Gene ID | 11093 |
| Gene name | ADAM metallopeptidase with thrombospondin type 1 motif 13 |
| Synonyms | ADAM-TS13|ADAMTS-13|C9orf8|VWFCP|vWF-CP |
| Cytomap | 9q34.2 |
| Type of gene | protein-coding |
| Description | A disintegrin and metalloproteinase with thrombospondin motifs 13a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13vWF-cleaving proteasevon Willebrand factor-cleaving protease |
| Modification date | 20240411 |
| UniProtAcc | Q76LX8 |
| Gene ontology of this gene with evidence of Inferred from Direct Assay (IDA) from Entrez |
| Partner | Gene | GO ID | GO term | PubMed ID |
| Gene | ADAMTS13 | GO:0006508 | proteolysis | 11535495 |
| Gene | ADAMTS13 | GO:0043171 | peptide catabolic process | 11535495 |
AS Summary |
| Information of the canonical protein with experimentally identified structure from PDB (2023). |
| UniProt Acc | File name | PDB ID | Method | Resolution | Chain | Start | End |
| Q76LX8-1 | Q76LX8-1_6qig_A.pdb | 6QIG | X-ray | 2.8 | A | 79 | 682 |
| ASpdb's canonical and alternatively spliced isoform information. |
| accession_id | gene_name | canonical_id | alternative_id | canonical_length | alternative_length | canonical_start | canonical_end | type | originalSEQ | variationSEQ | alternative_start | alternative_end |
| Q76LX8 | ADAMTS13 | Q76LX8-1 | Q76LX8-2 | 1427 | 1371 | 1135 | 1190 | Deletion | none | none | 1134 | 1134 |
| Q76LX8 | ADAMTS13 | Q76LX8-1 | Q76LX8-4 | 1427 | 364 | 2 | 329 | Deletion | none | none | 1 | 1 |
| Q76LX8 | ADAMTS13 | Q76LX8-1 | Q76LX8-4 | 1427 | 364 | 658 | 692 | Substitution | YRRYGEEYGNLTRPDITFTYFQPKPRQAWVWAAVR | GGVRAQLMHISWWSRPGLGERDLCARGRWPGGSSD | 330 | 364 |
| Q76LX8 | ADAMTS13 | Q76LX8-1 | Q76LX8-4 | 1427 | 364 | 693 | 1427 | Deletion | none | none | 364 | 364 |
| Multiple sequence alignment of our canonical and alternatively spliced ADAMTS13 |
| Matched gene isoform IDs with Ensembl and RefSeq of our canonical and alternative spliced genes of ADAMTS13 |
| UniProt-id | ENSG | ENST | ENSP |
| Q76LX8-1 | ENSG00000160323.19 | ENST00000371929.7 | ENSP00000360997.3 |
| Q76LX8-1 | ENSG00000281244.2 | ENST00000626597.2 | ENSP00000486201.1 |
| Q76LX8-2 | ENSG00000160323.19 | ENST00000355699.7 | ENSP00000347927.2 |
| Q76LX8-2 | ENSG00000281244.2 | ENST00000626744.2 | ENSP00000486734.1 |
| UniProt-id | NM ID | NP ID |
| Q76LX8-1 | NM_139025.4 | NP_620594.1 |
| Q76LX8-2 | NM_139027.4 | NP_620596.2 |
| Amino acid sequences of our canonical and alternatively spliced ADAMTS13 |
| accession_id | Protein sequence |
| Q76LX8-1 | MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRPPSPGFQRQRQRQRRAAGGILHLELLVAVGP DVFQAHQEDTERYVLTNLNIGAELLRDPSLGAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITR FDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGSGCGPSGHVMASDGAAPRAGLAWSPCSRRQL LSLLSAGRARCVWDPPRPQPGSAGHPPDAQPGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTEC GVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGGRACVGADLQAEMCNTQACEKTQLEFMSQQC ARTDGQPLRSSPGGASFYHWGAAVPHSQGDALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQ VWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIGGRYVVAGKMSISPNTTYPSLLEDGRVEYRV ALTEDRLPRLEEIRIWGPLQEDADIQVYRRYGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVE TVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTLPPARCRAGAQQPAVALETCNPQPCPARWEV SEPSSCTSAGGAGLALENETCVPGADGLEAPVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPA AGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLAACSVSCGRGVVRRILYCARAHGEDDGEEIL LDTQCQGLPRPEPQEACSLEPCPPRWKVMSLGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGT WMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGTPSLVPHEEAAAPGRTTATPAGASLEWSQARGLLFS PAPQPRRLLPGPQENSVQSSACGRQHLEPTGTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDM TFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATSNAGGCRLFINVAPHARIAIHALATNMGAGT |
| Q76LX8-2 | MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRPPSPGFQRQRQRQRRAAGGILHLELLVAVGP DVFQAHQEDTERYVLTNLNIGAELLRDPSLGAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITR FDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGSGCGPSGHVMASDGAAPRAGLAWSPCSRRQL LSLLSAGRARCVWDPPRPQPGSAGHPPDAQPGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTEC GVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGGRACVGADLQAEMCNTQACEKTQLEFMSQQC ARTDGQPLRSSPGGASFYHWGAAVPHSQGDALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQ VWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIGGRYVVAGKMSISPNTTYPSLLEDGRVEYRV ALTEDRLPRLEEIRIWGPLQEDADIQVYRRYGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVE TVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTLPPARCRAGAQQPAVALETCNPQPCPARWEV SEPSSCTSAGGAGLALENETCVPGADGLEAPVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPA AGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLAACSVSCGRGVVRRILYCARAHGEDDGEEIL LDTQCQGLPRPEPQEACSLEPCPPRWKVMSLGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGT WMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGACGRQHLEPTGTIDMRGPGQADCAVAIGRPLGEVVT LRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDMTFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLS PATSNAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWESESSQAEMEFSEGFLKAQASLRGQYW |
| Q76LX8-4 | MDMCQALSCHTDPLDQSSCSRLLVPLLDGTECGVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAF GGRACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGDALCRHMCRAIGESFIMKRGDSFLDGTRC MPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQVWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVR IGGRYVVAGKMSISPNTTYPSLLEDGRVEYRVALTEDRLPRLEEIRIWGPLQEDADIQVGGVRAQLMHISWWSRPGLGERDLCARGRWPG |
Protein Functional Features |
| Main function of this protein. (from UniProt) |
| ADAMTS13 (go to UniProt):Q76LX8 |
| Retention analysis result of protein across 39 protein features of UniProt such as six molecule processing features, 13 region features, four site features, six amino acid modification features, two natural variation features, five experimental info features, and 3 secondary structure features. Here, because of limited space for viewing, we only show the protein feature retention information belong to the 13 regional features. All retention annotation result can be downloaded at * Minus value of BPloci means that the break pointn is located before the CDS. |
| - Retained protein feature among the 13 regional features. |
| Accession_id | Subsection | Start | End | Funcitonal feature | Splicing information |
| Q76LX8 | Domain | 80 | 286 | Note=Peptidase M12B;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00276 | Type=Deletion;Start=2;End=329 |
| Q76LX8 | Domain | 287 | 383 | Note=Disintegrin | Type=Deletion;Start=2;End=329 |
| Q76LX8 | Domain | 682 | 730 | Note=TSP type-1 2;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Substitution;Start=658;End=692 |
| Q76LX8 | Domain | 682 | 730 | Note=TSP type-1 2;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 742 | 805 | Note=TSP type-1 3;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 808 | 859 | Note=TSP type-1 4;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 896 | 950 | Note=TSP type-1 5;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 951 | 1011 | Note=TSP type-1 6;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 1012 | 1068 | Note=TSP type-1 7;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 1072 | 1131 | Note=TSP type-1 8;Ontology_term=ECO:0000255;evidence=ECO:0000255|PROSITE-ProRule:PRU00210 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 1192 | 1298 | Note=CUB 1 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Domain | 1299 | 1427 | Note=CUB 2 | Type=Deletion;Start=693;End=1427 |
| Q76LX8 | Region | 51 | 70 | Note=Disordered;Ontology_term=ECO:0000256;evidence=ECO:0000256|SAM:MobiDB-lite | Type=Deletion;Start=2;End=329 |
| Q76LX8 | Region | 556 | 685 | Note=Spacer | Type=Substitution;Start=658;End=692 |
Gene Isoform Structures and Expression Levels for ADAMTS13 |
| Gene structures of our canonical and alternative spliced genes of ADAMTS13 * Click on the image to open the UCSC genome browser with custom track showing this image in a new window. |
| Expression levels of gene isoforms across GTEx. |
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| Expression levels of gene isoforms across TCGA. |
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Protein Structures |
| PDB and CIF files of the predicted protein structures * Here we show the 3D structure of the proteins using Mol*. AlphaFold produces a per-residue confidence score (pLDDT) between 0 and 100. Model confidence is shown from the pLDDT values per residue. pLDDT corresponds to the model’s prediction of its score on the local Distance Difference Test. It is a measure of local accuracy (from AlphfaFold website). To color code individual residues, we transformed individual PDB files into CIF format. |
| 3D view using mol* of Q76LX8-1 |
| 3D view using mol* of Q76LX8-2 |
| 3D view using mol* of Q76LX8-4 |
pLDDT Score Distribution |
| pLDDT score distribution of the predicted protein structures from AlphaFold2 * AlphaFold produces a per-residue confidence score (pLDDT) between 0 and 100. |
| pLDDT distribution across the protein length of Q76LX8-1 |
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| pLDDT distribution across the protein length of Q76LX8-2 |
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| pLDDT distribution across the protein length of Q76LX8-4 |
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Ramachandran Plot of Protein Structures |
| Ramachandran plot of the torsional angles - phi (φ)and psi (ψ) - of the residues (amino acids) contained in this protein peptide. |
| Ramachandran plot of Q76LX8-1 |
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| Ramachandran plot of Q76LX8-4 |
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Potential Active Site Information |
| The potential binding sites of these proteins were identified using SiteMap, a module of the Schrodinger suite. |
| UniProt-id | Site score | Size | D score | Volume | Exposure | Enclosure | Contact | Phobic | Philic | Balance | Don/Acc | Residues |
| Q76LX8-1 | 1.031 | 138 | 1.072 | 391.02 | 0.63 | 0.696 | 0.898 | 0.47 | 0.876 | 0.536 | 0.958 | 1212,1214,1221,1222,1223,1224,1243,1245,1246,1247, 1248,1250,1265,1266,1267,1270,1272,1291,1293,1294, 1295,1296,1300,1326,1328,1333,1334,1335,1336,1361, 1363,1364,1366,1382,1383,1384,1385,1387,1410 |
| Q76LX8-2 | 1.073 | 193 | 1.111 | 367.01 | 0.513 | 0.757 | 0.985 | 1.316 | 0.869 | 1.514 | 0.698 | 109,110,113,114,116,117,215,216,217,220,256,257,25 8,259,260,297,298,299,300,301,310,313,314,335,350, 352,353,354,355,356,366,367,368,369,380,381,382,38 3,384,385,386 |
| Q76LX8-4 | 1.012 | 312 | 0.995 | 739.165 | 0.53 | 0.716 | 0.951 | 0.538 | 1.15 | 0.467 | 0.728 | 70,73,74,104,107,109,110,112,113,114,117,150,179,1 81,182,183,184,185,186,187,188,189,190,191,192,203 ,204,206,214,215,216,218,224,253,254,255,256,271,2 89,290,292,293,294,295,296,297,298,299,316,317,318 ,319,320,321,322,323,342 |
Protein Structure and Feature Comparision |
| Protein Structure Comparision Using Template Modeling Scores (TM-score). |
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| Protein Structure Comparision Visualization with mol*. between Canonical predicted structure (AF2)(orange) vs Canonical validated structure (PDB)(green) |
| 3D view using mol* of Q76LX8-1_Q76LX8-1_6qig_A.pdb |
| Protein Structure Comparision Visualization with mol*. between Canonical validated structure (PDB)(orange) vs Alternative predicted structure (AF2)(green) |
| 3D view using mol* of Q76LX8-1_6qig_A_Q76LX8-2.pdb |
| 3D view using mol* of Q76LX8-1_6qig_A_Q76LX8-4.pdb |
| Protein Structure Comparision Visualization with mol*. between Canonical predicted structure (AF2)(orange) vs Alternative predicted structure (AF2)(green) |
| 3D view using mol* of Q76LX8-1_Q76LX8-2.pdb |
| 3D view using mol* of Q76LX8-1_Q76LX8-4.pdb |
| Protein Feature Comparison of the protein sequendary structures among the protiens. |
| ./stats/secondary_structure/figure/Q76LX8-1_vs_Q76LX8-2.png |
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| ./stats/secondary_structure/figure/Q76LX8-1_vs_Q76LX8-4.png |
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| Protein Feature Comparison of the relative accessible surface area (ASA) among the protiens. |
| ./stats/relative_asa/Q76LX8-1_vs_Q76LX8-2.png |
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| ./stats/relative_asa/Q76LX8-1_vs_Q76LX8-4.png |
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Protein-Protein Interaction |
| Interactors from UniProt. |
| Accession_id | Subsection | Start | End | Funcitonal feature | Splicing information |
| Interactors from STRING. |
| Gene name | Interactors |
Related Drugs to ADAMTS13 |
| Drugs targeting this gene/protein. (DrugBank) |
| UniProt accession | Gene name | DrugBank ID | Drug name | Drug group | Actions |
Related Diseases to ADAMTS13 |
| Previous studies relating to the alternative splicing of ADAMTS13 and disease information from the MeSH term (PubMed) |
| Gene | PMID | Title | Abstract | MeSH ID | MeSH term |
| ADAMTS13 | 20664912 | A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues. | Although ADAMTS13, the von Willebrand factor (VWF)-cleaving protease, is expressed in a range of tissues, the physiological significance of tissue-specific ADAMTS13 alternative splicing isoforms have yet to be clarified. Screening a panel of human tissues and cell lines revealed a spliced ADAMTS13 transcript in hepatic stellate cells and a hepatoma cell line that retains the 25th intron. A nonsense codon within the intron truncates the protease, which gains 64 novel amino acids in lieu of both CUB domains. This isoform, while retaining VWF-cleaving capability, accumulates intracellularly and its biological inaccessibility may prevent its participation in regulating haemostasis and other physiologic functions. | D006528 | Carcinoma, Hepatocellular |
| ADAMTS13 | 20664912 | A splice variant of ADAMTS13 is expressed in human hepatic stellate cells and cancerous tissues. | Although ADAMTS13, the von Willebrand factor (VWF)-cleaving protease, is expressed in a range of tissues, the physiological significance of tissue-specific ADAMTS13 alternative splicing isoforms have yet to be clarified. Screening a panel of human tissues and cell lines revealed a spliced ADAMTS13 transcript in hepatic stellate cells and a hepatoma cell line that retains the 25th intron. A nonsense codon within the intron truncates the protease, which gains 64 novel amino acids in lieu of both CUB domains. This isoform, while retaining VWF-cleaving capability, accumulates intracellularly and its biological inaccessibility may prevent its participation in regulating haemostasis and other physiologic functions. | D008113 | Liver Neoplasms |
Clinically important variants in ADAMTS13 |
| (ClinVar, 04/20/2024) |
| accession_id | uniprot_id | gene_name | Type | Variant | Clinical_significance |
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