Rabbit Anti-LTBP2/C14orf141/FITC Conjugated antibody

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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; L

Cat:

SL1888R-FITC

Species Reactivity：

(predicted: Human,Mouse,Rat,Pig,Cow,Horse,Rabbit,)

Immunogen：

KLH conjugated synthetic peptide derived from human LTBP2/C14orf141

Format：

Lyophilized or Liquid

Storage instructions：

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 ant

Buffer：

0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Concentration：

1mg/ml

Clonality：

Polyclonal

Isotype：

IgG

Applications：

ICC=1:50-200IF=1:50-200not yet tested in other applications.optimal dilutions/concentrations should be determined by the end user.

Host：

Rabbit

Calculated MW：

191kDa

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Unit:

Price: $596.00

Product PDFs

Datasheet: Down Datasheet

Other Information
Citations
Protein Attributes
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background:
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.

Database links:

Entrez Gene: 4053 Human

Omim: 602091 Human

SwissProt: Q14767 Human

Unigene: 512776 Human

Unigene: 597522 Human

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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