Product: ADAMTS13 Antibody
Catalog: DF9171
Description: Rabbit polyclonal antibody to ADAMTS13
Application: WB IHC IF/ICC
Reactivity: Human
Mol.Wt.: 154kDa; 154kD(Calculated).
Uniprot: Q76LX8
RRID: AB_2842367

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Product Info

Source:
Rabbit
Application:
IF/ICC 1:100-1:500, WB 1:1000-3000, IHC 1:50-1:200
*The optimal dilutions should be determined by the end user.
*Tips:

WB: For western blot detection of denatured protein samples. IHC: For immunohistochemical detection of paraffin sections (IHC-p) or frozen sections (IHC-f) of tissue samples. IF/ICC: For immunofluorescence detection of cell samples. ELISA(peptide): For ELISA detection of antigenic peptide.

Reactivity:
Human
Clonality:
Polyclonal
Specificity:
ADAMTS13 Antibody detects endogenous levels of total ADAMTS13.
RRID:
AB_2842367
Cite Format: Affinity Biosciences Cat# DF9171, RRID:AB_2842367.
Conjugate:
Unconjugated.
Purification:
The antiserum was purified by peptide affinity chromatography using SulfoLink™ Coupling Resin (Thermo Fisher Scientific).
Storage:
Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol. Store at -20 °C. Stable for 12 months from date of receipt.
Alias:

Fold/Unfold

A disintegrin and metalloproteinase with thrombospondin motifs 13; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif 13; A disintegrin like and metalloprotease with thrombospondin type 1 motif 13; ADAM metallopeptidase with thrombospondin type 1 motif 13; ADAM TS; ADAM-TS 13; ADAM-TS13; ADAMTS 13; ADAMTS-13; ADAMTS13; ADAMTS13 protein; ATS13_HUMAN; C9orf8; TTP; Von Willebrand factor cleaving protease; von Willebrand factor-cleaving protease; vWF cleaving protease; vWF CP; vWF-cleaving protease; vWF-CP; vWFCP;

Immunogens

Immunogen:
Uniprot:
Gene(ID):
Expression:
Q76LX8 ATS13_HUMAN:

Plasma. Expressed primarily in liver.

Sequence:
MHQRHPRARCPPLCVAGILACGFLLGCWGPSHFQQSCLQALEPQAVSSYLSPGAPLKGRPPSPGFQRQRQRQRRAAGGILHLELLVAVGPDVFQAHQEDTERYVLTNLNIGAELLRDPSLGAQFRVHLVKMVILTEPEGAPNITANLTSSLLSVCGWSQTINPEDDTDPGHADLVLYITRFDLELPDGNRQVRGVTQLGGACSPTWSCLITEDTGFDLGVTIAHEIGHSFGLEHDGAPGSGCGPSGHVMASDGAAPRAGLAWSPCSRRQLLSLLSAGRARCVWDPPRPQPGSAGHPPDAQPGLYYSANEQCRVAFGPKAVACTFAREHLDMCQALSCHTDPLDQSSCSRLLVPLLDGTECGVEKWCSKGRCRSLVELTPIAAVHGRWSSWGPRSPCSRSCGGGVVTRRRQCNNPRPAFGGRACVGADLQAEMCNTQACEKTQLEFMSQQCARTDGQPLRSSPGGASFYHWGAAVPHSQGDALCRHMCRAIGESFIMKRGDSFLDGTRCMPSGPREDGTLSLCVSGSCRTFGCDGRMDSQQVWDRCQVCGGDNSTCSPRKGSFTAGRAREYVTFLTVTPNLTSVYIANHRPLFTHLAVRIGGRYVVAGKMSISPNTTYPSLLEDGRVEYRVALTEDRLPRLEEIRIWGPLQEDADIQVYRRYGEEYGNLTRPDITFTYFQPKPRQAWVWAAVRGPCSVSCGAGLRWVNYSCLDQARKELVETVQCQGSQQPPAWPEACVLEPCPPYWAVGDFGPCSASCGGGLRERPVRCVEAQGSLLKTLPPARCRAGAQQPAVALETCNPQPCPARWEVSEPSSCTSAGGAGLALENETCVPGADGLEAPVTEGPGSVDEKLPAPEPCVGMSCPPGWGHLDATSAGEKAPSPWGSIRTGAQAAHVWTPAAGSCSVSCGRGLMELRFLCMDSALRVPVQEELCGLASKPGSRREVCQAVPCPARWQYKLAACSVSCGRGVVRRILYCARAHGEDDGEEILLDTQCQGLPRPEPQEACSLEPCPPRWKVMSLGPCSASCGLGTARRSVACVQLDQGQDVEVDEAACAALVRPEASVPCLIADCTYRWHVGTWMECSVSCGDGIQRRRDTCLGPQAQAPVPADFCQHLPKPVTVRGCWAGPCVGQGTPSLVPHEEAAAPGRTTATPAGASLEWSQARGLLFSPAPQPRRLLPGPQENSVQSSACGRQHLEPTGTIDMRGPGQADCAVAIGRPLGEVVTLRVLESSLNCSAGDMLLLWGRLTWRKMCRKLLDMTFSSKTNTLVVRQRCGRPGGGVLLRYGSQLAPETFYRECDMQLFGPWGEIVSPSLSPATSNAGGCRLFINVAPHARIAIHALATNMGAGTEGANASYILIRDTHSLRTTAFHGQQVLYWESESSQAEMEFSEGFLKAQASLRGQYWTLQSWVPEMQDPQSWKGKEGT

PTMs - Q76LX8 As Substrate

Site PTM Type Enzyme
T358 Phosphorylation
T435 Phosphorylation
N552 N-Glycosylation
N614 N-Glycosylation
N667 N-Glycosylation
S696 Phosphorylation
S698 O-Glycosylation
S698 Phosphorylation
N707 N-Glycosylation
S757 O-Glycosylation
S907 O-Glycosylation
S965 O-Glycosylation
S1027 O-Glycosylation
S1087 O-Glycosylation
T1294 Phosphorylation
Y1296 Phosphorylation
N1354 N-Glycosylation
S1356 Phosphorylation
Y1357 Phosphorylation

Research Backgrounds

Function:

Cleaves the vWF multimers in plasma into smaller forms thereby controlling vWF-mediated platelet thrombus formation.

PTMs:

Glycosylated. O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X(2)-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1,3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS13. May also be C-glycosylated on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and also N-glycosylated. These other glycosylations can also facilitate secretion.

The precursor is processed by a furin endopeptidase which cleaves off the pro-domain.

Subcellular Location:

Secreted.
Note: Secretion enhanced by O-fucosylation of TSP type-1 repeats.

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionSubcellular location
Tissue Specificity:

Plasma. Expressed primarily in liver.

Family&Domains:

The pro-domain is not required for folding or secretion and does not perform the common function of maintening enzyme latency.

The globular cysteineless spacer domain adopts a jelly-roll topology, and is necessary to recognize and cleave vWF. The C-terminal TSP type-1 and CUB domains may modulate this interaction.

References

1). ADAMTS13 regulates angiogenic markers via Ephrin/Eph signaling in human mesenchymal stem cells under serum-deprivation stress. Scientific reports, 2024 (PubMed: 38177376) [IF=4.6]

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