U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Links from GEO Profiles

    • Showing Current items.

    Adamts13 ADAM metallopeptidase with thrombospondin type 1 motif 13 [ Mus musculus (house mouse) ]

    Gene ID: 279028, updated on 30-Apr-2024

    Summary

    Go to the top of the page Help
    Official Symbol
    Adamts13provided by MGI
    Official Full Name
    ADAM metallopeptidase with thrombospondin type 1 motif 13provided by MGI
    Primary source
    MGI:MGI:2685556
    See related
    Ensembl:ENSMUSG00000014852 AllianceGenome:MGI:2685556
    Gene type
    protein coding
    RefSeq status
    REVIEWED
    Organism
    Mus musculus
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
    Also known as
    Gm710; vWF-CP; ADAM-TS13; ADAMTS-13
    Summary
    This gene encodes a member of "a disintegrin and metalloproteinase with thrombospondin motifs" (ADAMTS) family of multi-domain matrix-associated metalloendopeptidases that have diverse roles in tissue morphogenesis and pathophysiological remodeling, in inflammation and in vascular biology. In certain mouse strains (C57BL/6, for example) an intracisternal A-type particle (IAP) retrotransposon sequence is located in the intron 23 that causes an alternate splicing event resulting in a shorter transcript variants encoding shorter isoforms. The encoded preproprotein undergoes proteolytic processing to generate an active enzyme that cleaves von Willebrand factor (VWF) in circulating blood. [provided by RefSeq, Jul 2016]
    Expression
    Biased expression in liver adult (RPKM 2.2), liver E18 (RPKM 1.0) and 14 other tissues See more
    Orthologs
    human all
    NEW
    Try the new Gene table
    Try the new Transcript table

    Genomic context

    Go to the top of the page Help
    Location:
    2 A3; 2 19.14 cM
    Exon count:
    31
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCm39 (GCF_000001635.27) 2 NC_000068.8 (26863363..26899638)
    108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 2 NC_000068.7 (26973361..27009625)

    Chromosome 2 - NC_000068.8Genomic Context describing neighboring genes Neighboring gene surfeit gene 4 Neighboring gene STARR-positive B cell enhancer ABC_E2605 Neighboring gene serine/threonine kinase-like domain containing 1 Neighboring gene REX4, 3'-5' exonuclease Neighboring gene CapStarr-seq enhancer MGSCv37_chr2:26819876-26820059 Neighboring gene predicted gene, 36649 Neighboring gene STARR-positive B cell enhancer ABC_E7799 Neighboring gene calcium channel flower domain containing 1 Neighboring gene STARR-positive B cell enhancer ABC_E5901 Neighboring gene STARR-positive B cell enhancer ABC_E7800 Neighboring gene solute carrier family 2 (facilitated glucose transporter), member 6

    Genomic regions, transcripts, and products

    Go to the top of the page Help

    Go to nucleotide: Graphics FASTA GenBank

    Expression

    Go to the top of the page Help
    • Project title: Mouse ENCODE transcriptome data
    • Description: RNA profiling data sets generated by the Mouse ENCODE project.
    • BioProject: PRJNA66167
    • Publication: PMID 25409824
    • Analysis date: n/a

    Bibliography

    Go to the top of the pageHelp

    Related articles in PubMed

    1. Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function. Schelpe AS, et al. Blood Adv, 2020 Mar 24. PMID 32196558, Free PMC Article
    2. Formation and Resolution of Pial Microvascular Thrombosis in a Mouse Model of Thrombotic Thrombocytopenic Purpura. Adili R, et al. Arterioscler Thromb Vasc Biol, 2019 Sep. PMID 31340669, Free PMC Article
    3. ADAMTS13 Deficiency Shortens the Life Span of Mice With Experimental Diabetes. Cassis P, et al. Diabetes, 2018 Oct. PMID 29976618
    4. Long-Term Prevention of Congenital Thrombotic Thrombocytopenic Purpura in ADAMTS13 Knockout Mice by Sleeping Beauty Transposon-Mediated Gene Therapy. Verhenne S, et al. Arterioscler Thromb Vasc Biol, 2017 May. PMID 28254814
    5. N-acetylcysteine in preclinical mouse and baboon models of thrombotic thrombocytopenic purpura. Tersteeg C, et al. Blood, 2017 Feb 23. PMID 28011677

    See all (87) citations in PubMed

    GeneRIFs: Gene References Into Functions

    What's a GeneRIF?
    1. ADAMTS13 deficiency exacerbates neuroinflammation by targeting matrix metalloproteinase-9 in ischemic brain injury.
      Title: ADAMTS13 deficiency exacerbates neuroinflammation by targeting matrix metalloproteinase-9 in ischemic brain injury.
    2. Regulation of von Willebrand factor by ADAMTS13 ameliorates lipopolysaccharide-induced lung injury in mice.
      Title: Regulation of von Willebrand factor by ADAMTS13 ameliorates lipopolysaccharide-induced lung injury in mice.
    3. ADAMTS-13-regulated nuclear factor E2-related factor 2 signaling inhibits ferroptosis to ameliorate cisplatin-induced acute kidney injuy.
      Title: ADAMTS-13-regulated nuclear factor E2-related factor 2 signaling inhibits ferroptosis to ameliorate cisplatin-induced acute kidney injuy.
    4. Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function.
      Title: Antibodies that conformationally activate ADAMTS13 allosterically enhance metalloprotease domain function.
    5. Endothelial CD40 Mediates Microvascular von Willebrand Factor-Dependent Platelet Adhesion Inducing Inflammatory Venothrombosis in ADAMTS13 Knockout Mice.
      Title: Endothelial CD40 Mediates Microvascular von Willebrand Factor-Dependent Platelet Adhesion Inducing Inflammatory Venothrombosis in ADAMTS13 Knockout Mice.
    6. These results support a synergistic effect of severe ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy in mice.
      Title: Synergistic effects of ADAMTS13 deficiency and complement activation in pathogenesis of thrombotic microangiopathy.
    7. ADAMTS13 knockout promotes the occurrence of early brain injury after experimental subarachnoid hemorrhage.
      Title: Role of von Willebrand factor and ADAMTS-13 in early brain injury after experimental subarachnoid hemorrhage.
    8. ADAMTS13-vWF axis is partially involved in the pathophysiology of kidney ischemic reperfusion injury.
      Title: ADAMTS13-von Willebrand factor axis is involved in the pathophysiology of kidney ischaemia-reperfusion injury.
    9. Adamts13 deficiency in obese mice promotes hepatic microthrombosis.
      Title: ADAMTS13 deficiency promotes microthrombosis in a murine model of diet-induced liver steatosis.
    10. results suggest that ADAMTS13 controls key steps of ischemic vascular remodeling and that recombinant ADAMTS13 is a putative therapeutic avenue for promoting stroke recovery.
      Title: ADAMTS13 controls vascular remodeling by modifying VWF reactivity during stroke recovery.
    Submit: New GeneRIF Correction See all GeneRIFs (54)

    Variation

    Go to the top of the page Help

    Alleles

    Alleles of this type are documented at Mouse Genome Informatics  (MGI)

    General gene information

    Go to the top of the page Help

    Markers

    Homology

    Gene Ontology Provided by MGI

    Function Evidence Code Pubs
    enables endopeptidase activity IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    enables endopeptidase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables hydrolase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables metal ion binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables metalloendopeptidase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables metalloendopeptidase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    enables metallopeptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables peptidase activity ISO
    Inferred from Sequence Orthology
    more info
    		  
    Process Evidence Code Pubs
    acts_upstream_of_or_within blood coagulation IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in extracellular matrix organization IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    acts_upstream_of_or_within hemostasis IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in peptide catabolic process ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in protein catabolic process ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in proteolysis IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    involved_in proteolysis ISO
    Inferred from Sequence Orthology
    more info
    		  
    Component Evidence Code Pubs
    is_active_in extracellular matrix IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    located_in extracellular region IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in extracellular space ISO
    Inferred from Sequence Orthology
    more info
    		  

    General protein information

    Go to the top of the page Help
    Preferred Names
    A disintegrin and metalloproteinase with thrombospondin motifs 13
    Names
    a disintegrin-like and metallopeptidase (reprolysin type) with thrombospondin type 1 motif, 13
    a disintegrin-like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 13
    vWF-CP mRNA for von Willebrand factor-cleaving
    vWF-cleaving protease
    von Willebrand factor-cleaving protease
    NP_001001322.1
    NP_001277392.1
    NP_001277393.1
    NP_001277394.1
    XP_006498163.1
    XP_006498166.1
    XP_006498167.1

    NCBI Reference Sequences (RefSeq)

    Go to the top of the page Help

    NEW Try the new Transcript table

    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    mRNA and Protein(s)

    1. NM_001001322.2 → NP_001001322.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 1 preproprotein

      See identical proteins and their annotated locations for NP_001001322.1

      Status: REVIEWED

      Description
      Transcript Variant: This variant (1) represents the longest transcript and encodes the longest isoform (1).
      Source sequence(s)
      AB095445, AB112362
      Consensus CDS
      CCDS15820.1
      UniProtKB/Swiss-Prot
      A2ALB4, Q769J6, Q76LW1
      Related
      ENSMUSP00000099955.4, ENSMUST00000102891.4
      Conserved Domains (3) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
      pfam05986
      Location:565 → 683
      ADAM_spacer1; ADAM-TS Spacer 1

    2. NM_001290463.1 → NP_001277392.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform 2 precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (2) uses an alternate in-frame splice site in the central coding region, compared to variant 1, resulting in an isoform (2) that is shorter than isoform 1. This isoform (2) may undergo proteolytic processing similar to isoform 1.
      Source sequence(s)
      AB095445, AB112362
      UniProtKB/Swiss-Prot
      Q769J6
      Conserved Domains (3) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
      pfam05986
      Location:565 → 683
      ADAM_spacer1; ADAM-TS Spacer 1

    3. NM_001290464.1 → NP_001277393.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform IAP-a precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (IAP-a) lacks seven 3' exons but instead contains an alternate 3' terminal exon, and it thus differs in the 3' coding region and 3' UTR, compared to variant 1. The encoded isoform (IAP-a) has a distinct C-terminus and is shorter than isoform 1. This isoform (IAP-a) may undergo proteolytic processing similar to isoform 1.
      Source sequence(s)
      AL773563, AL845266
      Consensus CDS
      CCDS71010.1
      UniProtKB/TrEMBL
      A2ALB3, A7LM19
      Related
      ENSMUSP00000014996.8, ENSMUST00000014996.14
      Conserved Domains (3) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
      pfam05986
      Location:565 → 683
      ADAM_spacer1; ADAM-TS Spacer 1

    4. NM_001290465.1 → NP_001277394.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform IAP-b precursor

      Status: REVIEWED

      Description
      Transcript Variant: This variant (IAP-b) uses an alternate in-frame splice site and lacks an alternate in-frame exon in the central coding region, and it also lacks seven 3' exons but instead contains an alternate 3' terminal exon and thus differs in the 3' coding region and 3' UTR, compared to variant 1. The encoded isoform (IAP-b) has a distinct C-terminus and is shorter than isoform 1. This isoform (IAP-b) may undergo proteolytic processing similar to isoform 1.
      Source sequence(s)
      AL773563, AL845266, EU034706
      UniProtKB/TrEMBL
      A7LM19
      Conserved Domains (2) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...

    RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCm39 C57BL/6J

    Genomic

    1. NC_000068.8 Reference GRCm39 C57BL/6J

      Range
      26863363..26899638
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_006498100.5 → XP_006498163.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform X1

      Conserved Domains (3) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
      pfam17771
      Location:306 → 376
      ADAM_CR_2; ADAM cysteine-rich domain

    2. XM_006498104.5 → XP_006498167.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform X3

      UniProtKB/TrEMBL
      A7LM19
      Conserved Domains (4) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
      pfam05986
      Location:565 → 683
      ADAM_spacer1; ADAM-TS Spacer 1
      pfam17771
      Location:306 → 376
      ADAM_CR_2; ADAM cysteine-rich domain

    3. XM_006498103.5 → XP_006498166.1  A disintegrin and metalloproteinase with thrombospondin motifs 13 isoform X2

      UniProtKB/TrEMBL
      A7LM19
      Conserved Domains (4) summary
      smart00209
      Location:392 → 444
      TSP1; Thrombospondin type 1 repeats
      cd04273
      Location:82 → 286
      ZnMc_ADAMTS_like; Zinc-dependent metalloprotease, ADAMTS_like subgroup. ADAMs (A Disintegrin And Metalloprotease) are glycoproteins, which play roles in cell signaling, cell fusion, and cell-cell interactions. This particular subfamily represents domain architectures that ...
      pfam05986
      Location:565 → 683
      ADAM_spacer1; ADAM-TS Spacer 1
      pfam17771
      Location:306 → 376
      ADAM_CR_2; ADAM cysteine-rich domain

    RNA

    1. XR_001782991.3 RNA Sequence

    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q769J6.1 GenPept UniProtKB/Swiss-Prot:Q769J6

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

    Chemical Information
    Molecular Biology Databases
    Research Materials
    Tools
    Miscellaneous