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Product Name ADAMTSL4 Chinese Name 整合素样金属蛋白酶与凝血酶样4蛋白抗体 Alias ADAMTS like 4; ADAMTS like protein 4; ADAMTSL-4; ADAMTSL 4; Thrombospondin repeat containing 1; thrombospondin repeat protein 1; TSRC1; ATL4_HUMAN. Research Area Tumour Cell biology immunology Signal transduction Cell adhesion molecule Immunogen Species Rabbit Clonality Polyclonal React Species Mouse, (predicted: Human, Rat, Dog, Pig, Cow, Horse, Sheep, ) Applications WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=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 114kDa Cellular localization Extracellular matrix Secretory protein Form Liquid Concentration 1mg/ml immunogen KLH conjugated synthetic peptide derived from human ADAMTSL4: 351-450/1074 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 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
Extracellular matrix蛋白Product Picture Sample:
Lung (Mouse) Lysate at 40 ug
Primary: Anti- ADAMTSL4 (SL5864R) 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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