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Rabbit Anti-ADAMTS10 /BF594 Conjugated antibody (bs-23478R-BF594)
訂購熱線:400-901-9800
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訂購QQ:  400-901-9800
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-23478R-BF594
英文名稱 Rabbit Anti-ADAMTS10 /BF594 Conjugated antibody
中文名稱 BF594標記的整合素樣金屬蛋白酶與凝血酶10型抗體
別    名 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.  
規格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 細胞生物  信號轉導  細胞外基質  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 ICC=1:50-200 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 95kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ADAMTS10
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 background:
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.

Database links:

Entrez Gene: 81794 Human

Entrez Gene: 224697 Mouse

Entrez Gene: 314655 Rat

Omim: 608990 Human

SwissProt: Q9H324 Human

SwissProt: P58459 Mouse

Unigene: 657508 Human

Unigene: 29304 Mouse

Unigene: 135600 Rat



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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