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ADAMTSL4 Rabbit pAb (bs-5864R)  
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產品編號 bs-5864R
英文名稱 ADAMTSL4 Rabbit pAb
中文名稱 整合素樣金屬蛋白酶與凝血酶樣4蛋白抗體
別    名 ADAMTS like 4; ADAMTS like protein 4; ADAMTSL-4; ADAMTSL 4; Thrombospondin repeat containing 1; thrombospondin repeat protein 1; TSRC1; ATL4_HUMAN.  
研究領域 腫瘤  細胞生物  免疫學  信號轉導  細胞粘附分子  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Mouse (predicted: Human,Rat,Pig,Sheep,Cow,Dog,Horse)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 114 kDa
檢測分子量
細胞定位 細胞外基質 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human ADAMTSL4: 351-450/1074 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 ADAMTSL4 is a member of ADAMTS (a disintegrin and metalloproteinase with thrombospondin motifs)-like family and has seven thrombospondin type 1 repeats. The thrombospondin type 1 repeat domain is found in many proteins with diverse biological functions including cellular adhesion, angiogenesis, and patterning of the developing nervous system. Alternate transcriptional splice variants, encoding different isoforms, have been characterized. ADAMTSL4 is involved in the positive regulation of apoptosis.

Function:
Positive regulation of apoptosis. May facilitate FBN1 microfibril biogenesis.

Subunit:
Interacts with CTSB. Interacts with FBN1.

Subcellular Location:
Secreted, extracellular space, extracellular matrix. Note=Colocalizes with FMN1 microfibrils in the eye ECM.

Tissue Specificity:
Expressed in colon, heart, leukocyte, liver, lung, skeletal muscle, spleen, testis and placenta. Weaker expression in bone marrow, brain tissue, kidney and pancreas. Expression studies in fetal tissues reveal strong expression in heart, kidney, liver, lung and skeletal muscle, but weaker expression in fetal brain and skin.

Post-translational modifications:
N-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. N- and C-glycosylations can also facilitate secretion (By similarity).

DISEASE:
Defects in ADAMTSL4 are a cause of ectopia lentis, isolated, autosomal recessive (ECTOL2) [MIM:225100]. A rare condition characterized by partial or complete displacement of the lens from its space resulting from defective zonule formation.

Similarity:
Contains 1 PLAC domain.
Contains 6 TSP type-1 domains.

SWISS:
Q6UY14

Gene ID:
54507

Database links:

Entrez Gene: 54507 Human

Omim: 610113 Human

SwissProt: Q6UY14 Human



細胞外基質蛋白
產品圖片
Sample: Lung (Mouse) Lysate at 40 ug Primary: Anti- ADAMTSL4 (bs-5864R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 114 kD Observed band size: 124 kD
Sample: Heart (Mouse) Lysate at 40 ug Primary: Anti- ADAMTSL4 (bs-5864R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 114 kD Observed band size: 124 kD
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