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LTBP2/C14orf141 Rabbit pAb (bs-18440R)  
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產品編號 bs-18440R
英文名稱 LTBP2/C14orf141 Rabbit pAb
中文名稱 潛在型TGF-β結合蛋白2抗體
別    名 C14orf141; Chromosome 14 open reading frame 141; Latent TGF beta binding protein 2; Latent transforming growth factor beta binding protein 2; Latent-transforming growth factor beta-binding protein 2; LTBP 2; LTBP 3; LTBP-2; Ltbp2; LTBP2_HUMAN; LTBP3; MSTP 031; MSTP031.  
Specific References  (2)     |     bs-18440R has been referenced in 2 publications.
[IF=3.258] Hang Li. et al. Identification of Potential Pathogenic Super-Enhancers-Driven Genes in Pulmonary Fibrosis. Front Genet. 2021; 12: 644143  IF ;  Mouse.  
[IF=0] William J. Burlingham. et al. Extracellular Vesicle–associated GARP/TGFβ:LAP Mediates “Infectious” Allo-tolerance. TRANSPLANT DIRECT. 2023 Jun; 9(6): e1475  ELISA ;  Mouse.  
研究領域 細胞生物  免疫學  信號轉導  生長因子和激素  結合蛋白  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 (predicted: Human,Mouse,Rat,Rabbit,Pig,Cow,Horse)
產品應用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ICC/IF=1:100-500,ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 191 kDa
檢測分子量
細胞定位 分泌型蛋白 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LTBP2/C14orf141: 701-800/1821 
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 The protein encoded by this gene belongs to the family of latent transforming growth factor (TGF)-beta binding proteins (LTBP), which are extracellular matrix proteins with multi-domain structure. This protein is the largest member of the LTBP family possessing unique regions and with most similarity to the fibrillins. It has thus been suggested that it may have multiple functions: as a member of the TGF-beta latent complex, as a structural component of microfibrils, and a role in cell adhesion. [provided by RefSeq, Jul 2008]

Function:
May play an integral structural role in elastic-fiber architectural organization and/or assembly.

Subcellular Location:
Secreted. Localized in nuchal ligament and aorta to the fibrillin-containing, microfibrillar component of elastic fibers.

Tissue Specificity:
Expressed in lung, weakly expressed in heart, placenta, liver and skeletal muscle.

Post-translational modifications:
Contains hydroxylated asparagine residues.

DISEASE:
Defects in LTBP2 are the cause of primary congenital glaucoma type 3D (GLC3D) [MIM:613086]. An autosomal recessive form of primary congenital glaucoma (PCG). PCG is characterized by marked increase of intraocular pressure at birth or early childhood, large ocular globes (buphthalmos) and corneal edema. It results from developmental defects of the trabecular meshwork and anterior chamber angle of the eye that prevent adequate drainage of aqueous humor.
Defects in LTBP2 are the cause of microspherophakia (MCSPH) [MIM:251750]. It is a rare disease characterized by smaller and more spherical lenses than normal bilaterally, an increased anteroposterior thickness of the lens and highly myopic eyes. Lens dislocation or subluxation may occur, leading to defective accommodation.

Similarity:
Belongs to the LTBP family.
Contains 20 EGF-like domains.
Contains 4 TB (TGF-beta binding) domains.

SWISS:
Q14767

Gene ID:
4053

Database links:

Entrez Gene: 4053 Human

Omim: 602091 Human

SwissProt: Q14767 Human

Unigene: 512776 Human

Unigene: 597522 Human



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