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Product Name ADAMTS10 Chinese Name 整合素样金属蛋白酶与凝血酶10型抗体 Alias A disintegrin and metalloproteinase with thrombospondin motifs 10; A disintegrin like and metalloprotease (reprolysin type) with thrombospondin type 1 motif, 10; ADAM metallopeptidase with thrombospondin type 1 motif 10; ADAM TS10; EC 3.4.24; WMS; ATS10_HUMAN. Research Area Cell biology Signal transduction Extracellular matrix Immunogen Species Rabbit Clonality Polyclonal React Species Rat, (predicted: Human, Mouse, Pig, Cow, Horse, Rabbit, Sheep, ) Applications WB=1:500-2000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 (Paraffin sections need antigen repair)
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.Theoretical molecular weight 95kDa Cellular localization Extracellular matrix Secretory protein Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human ADAMTS10 : 431-530/1103 Lsotype IgG Purification affinity purified by Protein A Buffer Solution 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Storage Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. Attention This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. PubMed PubMed Product Detail ADAMTS10 is a member of the ADAMs family of proteinases with Thrombospondin motifs. The catalytic site of ADAMTS10 is typical of the metalloproteinase catalytic domains, with an HExxHxxxxxH sequence, perhaps giving these enzymes some shared specificity. ADAMTS10 is closest in homology to ADAMTS6, sharing 53% overall identity. Functional mutations in ADAMTS10 have been linked to Weill Marchesani syndrome, a connective tissue disorder marked by fibrillin 1 misprocessing. ADAMTS10 has also been reported to be over expressed in breast cancer tissues and cell lines.
Function:
Metalloprotease that participate in microfibrils assembly. Microfibrils are extracellular matrix components occurring independently or along with elastin in the formation of elastic tissues.
Subunit:
Interacts with FBN1; this interaction promotes microfibrils assembly.
Subcellular Location:
Secreted, extracellular space, extracellular matrix.
Tissue Specificity:
Widely expressed in adult tissues.
Post-translational modifications:
Glycosylated. Can be 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 ADAMTS family members. Also can be C-glycosylated with one or two mannose molecules on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and N-glycosylated. These other glycosylations can also facilitate secretion (By similarity).
DISEASE:
Defects in ADAMTS10 are the cause of Weill-Marchesani syndrome 1 (WMS1) [MIM:277600]. WMS1 is a rare connective tissue disorder characterized by short stature, brachydactyly, joint stiffness, and eye abnormalities including microspherophakia, ectopia lentis, severe myopia and glaucoma.
Similarity:
Contains 1 disintegrin domain.
Contains 1 peptidase M12B domain.
Contains 1 PLAC domain.
Contains 5 TSP type-1 domains.
SWISS:
Q9H324
Gene ID:
81794
Database links:Entrez Gene: 81794 Human
Entrez Gene: 224697 Mouse
Omim: 608990 Human
SwissProt: Q9H324 Human
SwissProt: P58459 Mouse
Unigene: 657508 Human
Unigene: 29304 Mouse
Unigene: 135600 Rat
Product Picture Paraformaldehyde-fixed, paraffin embedded (rat brain tissue); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (ADAMTS10) Polyclonal Antibody, Unconjugated (SL23478R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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