LTBP4 Rabbit pAb
LTBP4 Rabbit pAb
- 产品详情
- 实验流程
- 背景知识
Application
| WB, IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | Q8N2S1 |
| Reactivity | Rat, Pig, Mouse, Dog |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 173435 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human LTBP4 |
| Epitope Specificity | 151-250/1624 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Secreted, extracellular space, extracellular matrix. |
| SIMILARITY | Belongs to the LTBP family.Contains 16 EGF-like domains.Contains 4 TB (TGF-beta binding) domains. |
| SUBUNIT | Forms part of the large latent transforming growth factor beta precursor complex; removal is essential for activation of complex. Interacts with LTBP1 and TGFB1. Binds to FBN1 (By similarity). |
| Post-translational modifications | Contains hydroxylated asparagine residues (By similarity). |
| DISEASE | Defects in LTBP4 are the cause of Urban-Rifkin-Davis syndrome (URDS) [MIM:613177]; also known as Cutis laxa with severe pulmonary gastrointestinal and urinary abnormalities. URDS is a syndrome characterized by disrupted pulmonary, gastrointestinal, urinary, musculoskeletal, craniofacial and dermal development. Clinical features include cutis laxa, mild cardiovascular lesions, respiratory distress with cystic and atelectatic changes in the lungs, and diverticulosis, tortuosity and stenosis at various levels of the intestinal tract. Craniofacial features include microretrognathia, flat midface, receding forehead and wide fontanelles. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | May be involved in the assembly, secretion and targeting of TGFB1 to sites at which it is stored and/or activated. May play critical roles in controlling and directing the activity of TGFB1. May have a structural role in the extra cellular matrix (ECM). |
| Gene ID | 8425 |
|---|---|
| Other Names | Latent-transforming growth factor beta-binding protein 4, LTBP-4, LTBP4 |
| Target/Specificity | Highly expressed in heart, skeletal muscle, pancreas, uterus, and small intestine. Weakly expressed in placenta and lung. |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
| Name | LTBP4 |
|---|---|
| Function | Key regulator of transforming growth factor beta (TGFB1, TGFB2 and TGFB3) that controls TGF-beta activation by maintaining it in a latent state during storage in extracellular space. Associates specifically via disulfide bonds with the Latency-associated peptide (LAP), which is the regulatory chain of TGF-beta, and regulates integrin-dependent activation of TGF-beta. |
| Cellular Location | Secreted, extracellular space, extracellular matrix |
| Tissue Location | Highly expressed in heart, skeletal muscle, pancreas, uterus, and small intestine. Weakly expressed in placenta and lung. |
Research Areas
For Research Use Only. Not For Use In Diagnostic Procedures.
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
BACKGROUND
May be involved in the assembly, secretion and targeting of TGFB1 to sites at which it is stored and/or activated. May play critical roles in controlling and directing the activity of TGFB1. May have a structural role in the extra cellular matrix (ECM).
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