KLH conjugated synthetic peptide derived from human LTBP2/C14orf141:701-800/1821
Format：
Liquid
Storage instructions：
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Concentration：
1mg/ml
Clonality：
Polyclonal
Isotype：
IgG
Applications：
ELISA=1:5000-10000IHC-P=1:100-500IHC-F=1:100-500ICC=1:100-500IF=1:100-500（Paraffin sections need to do antigen repair）not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.
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.
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.
Specific References (1) | SL1888R has been referenced in 1 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.
