KEGG   Homo sapiens (human): 11093
Entry
11093             CDS       T01001                                 
Symbol
ADAMTS13, ADAM-TS13, ADAMTS-13, C9orf8, VWFCP, vWF-CP
Name
(RefSeq) ADAM metallopeptidase with thrombospondin type 1 motif 13
  KO
K08627  a disintegrin and metalloproteinase with thrombospondin motifs 13 [EC:3.4.24.87]
Organism
hsa  Homo sapiens (human)
Pathway
hsa04611  Platelet activation
Network
nt06514  Coagulation cascade
  Element
N01831  Regulation of VWF-GPIb-IX-V interaction, ADAMTS13
Disease
H00225  Thrombotic thrombocytopenic purpura
Drug target
Apadamtase alfa: D12569
Apadamtase alfa and cinaxadamtase alfa: D12835<JP/US>
Cinaxadamtase alfa: D12570
Brite
KEGG Orthology (KO) [BR:hsa00001]
 09150 Organismal Systems
  09151 Immune system
   04611 Platelet activation
    11093 (ADAMTS13)
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01002 Peptidases and inhibitors [BR:hsa01002]
    11093 (ADAMTS13)
Enzymes [BR:hsa01000]
 3. Hydrolases
  3.4  Acting on peptide bonds (peptidases)
   3.4.24  Metalloendopeptidases
    3.4.24.87  ADAMTS13 endopeptidase
     11093 (ADAMTS13)
Peptidases and inhibitors [BR:hsa01002]
 Metallo peptidases
  Family M12: astacin/adamalysin family
   11093 (ADAMTS13)
SSDB
Motif
Pfam: ADAMTS_CR_3 TSP1_ADAMTS ADAMTS_spacer1 TSP_1 ADAMTS_CR_2 Reprolysin Reprolysin_4 Reprolysin_3 TSP1_spondin Reprolysin_5 Peptidase_M10 Reprolysin_2
Other DBs
NCBI-GeneID: 11093
NCBI-ProteinID: NP_620594
OMIM: 604134
HGNC: 1366
Ensembl: ENSG00000160323
UniProt: Q76LX8
Structure
Position
9:133414337..133459386
AA seq 1427 aa
MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRP
PSPGFQRQRQRQRRAAGGILHLELLVAVGPDVFQAHQEDTERYVLTNLNIGAELLRDPSL
GAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITR
FDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGS
GCGPSGHVMASDGAAPRAGLAWSPCSRRQLLSLLSAGRARCVWDPPRPQPGSAGHPPDAQ
PGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTEC
GVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGG
RACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGD
ALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQ
VWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIG
GRYVVAGKMSISPNTTYPSLLEDGRVEYRVALTEDRLPRLEEIRIWGPLQEDADIQVYRR
YGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVE
TVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTL
PPARCRAGAQQPAVALETCNPQPCPARWEVSEPSSCTSAGGAGLALENETCVPGADGLEA
PVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPA
AGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLA
ACSVSCGRGVVRRILYCARAHGEDDGEEILLDTQCQGLPRPEPQEACSLEPCPPRWKVMS
LGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGT
WMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGTPSLVP
HEEAAAPGRTTATPAGASLEWSQARGLLFSPAPQPRRLLPGPQENSVQSSACGRQHLEPT
GTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDM
TFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATS
NAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWE
SESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT
NT seq 4284 nt   +upstreamnt  +downstreamnt
atgcaccagcgtcacccccgggcaagatgccctcccctctgtgtggccggaatccttgcc
tgtggctttctcctgggctgctggggaccctcccatttccagcagagttgtcttcaggct
ttggagccacaggccgtgtcttcttacttgagccctggtgctcccttaaaaggccgccct
ccttcccctggcttccagaggcagaggcagaggcagaggcgggctgcaggcggcatccta
cacctggagctgctggtggccgtgggccccgatgtcttccaggctcaccaggaggacaca
gagcgctatgtgctcaccaacctcaacatcggggcagaactgcttcgggacccgtccctg
ggggctcagtttcgggtgcacctggtgaagatggtcattctgacagagcctgagggtgct
ccaaatatcacagccaacctcacctcgtccctgctgagcgtctgtgggtggagccagacc
atcaaccctgaggacgacacggatcctggccatgctgacctggtcctctatatcactagg
tttgacctggagttgcctgatggtaaccggcaggtgcggggcgtcacccagctgggcggt
gcctgctccccaacctggagctgcctcattaccgaggacactggcttcgacctgggagtc
accattgcccatgagattgggcacagcttcggcctggagcacgacggcgcgcccggcagc
ggctgcggccccagcggacacgtgatggcttcggacggcgccgcgccccgcgccggcctc
gcctggtccccctgcagccgccggcagctgctgagcctgctcagcgcaggacgggcgcgc
tgcgtgtgggacccgccgcggcctcaacccgggtccgcggggcacccgccggatgcgcag
cctggcctctactacagcgccaacgagcagtgccgcgtggccttcggccccaaggctgtc
gcctgcaccttcgccagggagcacctggatatgtgccaggccctctcctgccacacagac
ccgctggaccaaagcagctgcagccgcctcctcgttcctctcctggatgggacagaatgt
ggcgtggagaagtggtgctccaagggtcgctgccgctccctggtggagctgacccccata
gcagcagtgcatgggcgctggtctagctggggtccccgaagtccttgctcccgctcctgc
ggaggaggtgtggtcaccaggaggcggcagtgcaacaaccccagacctgcctttgggggg
cgtgcatgtgttggtgctgacctccaggccgagatgtgcaacactcaggcctgcgagaag
acccagctggagttcatgtcgcaacagtgcgccaggaccgacggccagccgctgcgctcc
tcccctggcggcgcctccttctaccactggggtgctgctgtaccacacagccaaggggat
gctctgtgcagacacatgtgccgggccattggcgagagcttcatcatgaagcgtggagac
agcttcctcgatgggacccggtgtatgccaagtggcccccgggaggacgggaccctgagc
ctgtgtgtgtcgggcagctgcaggacatttggctgtgatggtaggatggactcccagcag
gtatgggacaggtgccaggtgtgtggtggggacaacagcacgtgcagcccacggaagggc
tctttcacagctggcagagcgagagaatatgtcacgtttctgacagttacccccaacctg
accagtgtctacattgccaaccacaggcctctcttcacacacttggcggtgaggatcgga
gggcgctatgtcgtggctgggaagatgagcatctcccctaacaccacctacccctccctc
ctggaggatggtcgtgtcgagtacagagtggccctcaccgaggaccggctgccccgcctg
gaggagatccgcatctggggacccctccaggaagatgctgacatccaggtttacaggcgg
tatggcgaggagtatggcaacctcacccgcccagacatcaccttcacctacttccagcct
aagccacggcaggcctgggtgtgggccgctgtgcgtgggccctgctcggtgagctgtggg
gcagggctgcgctgggtaaactacagctgcctggaccaggccaggaaggagttggtggag
actgtccagtgccaagggagccagcagccaccagcgtggccagaggcctgcgtgctcgaa
ccctgccctccctactgggcggtgggagacttcggcccatgcagcgcctcctgtgggggt
ggcctgcgggagcggccagtgcgctgcgtggaggcccagggcagcctcctgaagacattg
cccccagcccggtgcagagcaggggcccagcagccagctgtggcgctggaaacctgcaac
ccccagccctgccctgccaggtgggaggtgtcagagcccagctcatgcacatcagctggt
ggagcaggcctggccttggagaacgagacctgtgtgccaggggcagatggcctggaggct
ccagtgactgaggggcctggctccgtagatgagaagctgcctgcccctgagccctgtgtc
gggatgtcatgtcctccaggctggggccatctggatgccacctctgcaggggagaaggct
ccctccccatggggcagcatcaggacgggggctcaagctgcacacgtgtggacccctgcg
gcagggtcgtgctccgtctcctgcgggcgaggtctgatggagctgcgtttcctgtgcatg
gactctgccctcagggtgcctgtccaggaagagctgtgtggcctggcaagcaagcctggg
agccggcgggaggtctgccaggctgtcccgtgccctgctcggtggcagtacaagctggcg
gcctgcagcgtgagctgtgggagaggggtcgtgcggaggatcctgtattgtgcccgggcc
catggggaggacgatggtgaggagatcctgttggacacccagtgccaggggctgcctcgc
ccggaaccccaggaggcctgcagcctggagccctgcccacctaggtggaaagtcatgtcc
cttggcccatgttcggccagctgtggccttggcactgctagacgctcggtggcctgtgtg
cagctcgaccaaggccaggacgtggaggtggacgaggcggcctgtgcggcgctggtgcgg
cccgaggccagtgtcccctgtctcattgccgactgcacctaccgctggcatgttggcacc
tggatggagtgctctgtttcctgtggggatggcatccagcgccggcgtgacacctgcctc
ggaccccaggcccaggcgcctgtgccagctgatttctgccagcacttgcccaagccggtg
actgtgcgtggctgctgggctgggccctgtgtgggacagggtacgcccagcctggtgccc
cacgaagaagccgctgctccaggacggaccacagccacccctgctggtgcctccctggag
tggtcccaggcccggggcctgctcttctccccggctccccagcctcggcggctcctgccc
gggccccaggaaaactcagtgcagtccagtgcctgtggcaggcagcaccttgagccaaca
ggaaccattgacatgcgaggcccagggcaggcagactgtgcagtggccattgggcggccc
ctcggggaggtggtgaccctccgcgtccttgagagttctctcaactgcagtgcgggggac
atgttgctgctttggggccggctcacctggaggaagatgtgcaggaagctgttggacatg
actttcagctccaagaccaacacgctggtggtgaggcagcgctgcgggcggccaggaggt
ggggtgctgctgcggtatgggagccagcttgctcctgaaaccttctacagagaatgtgac
atgcagctctttgggccctggggtgaaatcgtgagcccctcgctgagtccagccacgagt
aatgcagggggctgccggctcttcattaatgtggctccgcacgcacggattgccatccat
gccctggccaccaacatgggcgctgggaccgagggagccaatgccagctacatcttgatc
cgggacacccacagcttgaggaccacagcgttccatgggcagcaggtgctctactgggag
tcagagagcagccaggctgagatggagttcagcgagggcttcctgaaggctcaggccagc
ctgcggggccagtactggaccctccaatcatgggtaccggagatgcaggaccctcagtcc
tggaagggaaaggaaggaacctga

  All links  
Disease (2)   
   OMIM (2)   
Gene (45)   
   RefGene (40)   
   NCBI-PROTEINID (1)   
   NCBI-Gene (1)   
   HGNC (1)   
   Ensembl (1)   
   OC (1)   
Protein sequence (30)   
   UniProt (1)   
   SWISS-PROT (1)   
   RefSeq(pep) (28)   
DNA sequence (70)   
   RefSeq(nuc) (30)   
   GenBank (20)   
   EMBL (20)   
3D Structure (5)   
   PDB (5)   
Protein domain (12)   
   Pfam (12)   
All databases (164)   

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