Anti-ADAMTSL4抗体β-淀粉样蛋白来自β-淀粉样蛋白原，在脑组织的细胞外呈丝状蛋白样沉积 ，是淀粉样结节性神经炎病变的主要蛋白成分，在神经纤维中也有沉积。

产品编号 yb-5864R
英文名称 Anti-ADAMTSL4抗体
中文名称 整合素样金属蛋白酶与*样4蛋白抗体
别    名 ADAMTS like 4; ADAMTS like protein 4; ADAMTSL-4; ADAMTSL 4; Thrombospondin repeat containing 1; thrombospondin repeat protein 1; TSRC1; ATL4_HUMAN.
 Anti-ADAMTSL4抗体  
说 明 书 0.2ml  
研究领域 肿瘤  细胞生物  免疫学  信号转导  细胞粘附分子  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, 
产品应用 WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 114kDa
细胞定位 细胞外基质 分泌型蛋白 
性    状 Lyophilized or Liquid
浓    度 1mg/1ml
免 疫 原 KLH conjugated synthetic peptide derived from human ADAMTSL4
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
 
PubMed PubMed
产品介绍 background:
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. 

Database links:
UniProtKB/Swiss-Prot: Q6UY14.2
 
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 

细胞外基质蛋白