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LTBP3 (show LTBP2 ELISA Kits) is a novel component of the microfibrillar network involved in the acromelic dysplasia spectrum.
New recessive truncating mutation in LTBP3 (show LTBP2 ELISA Kits) has been described in a family with oligodontia, short stature, and mitral valve prolapse.
Phenotype-genotype correlations between LTBP3 (show LTBP2 ELISA Kits) mutations and families with brachyolmia with amelogenesis imperfecta.
the mechanism of transcriptional activation of LTBP3 (show LTBP2 ELISA Kits) promoter depends on MALAT1 initiated from neighboring gene LTBP3 (show LTBP2 ELISA Kits) and involves both the direct interaction of the Sp1 (show PSG1 ELISA Kits) and promoter-specific activation
Latent transforming growth factor beta-binding proteins-2 and -3 inhibit the proprotein convertase 5 (show PCSK5 ELISA Kits)/6A.
These findings indicate that human latent TGF-beta-binding protein-3 (LTBP-3) has an essential role in the secretion and targeting of transforming growth factor-beta1 (TGF-beta1 (show TGFB1 ELISA Kits)).
Extracellular matrix is an important site of deposition for LTBP-3 (show LTBP2 ELISA Kits) and LTBP-4 (show LTBP4 ELISA Kits).
stimulation of transcriptional activity of promoter is stimulated by TGF-beta 1 (show TGFB1 ELISA Kits)
Data show that LTBP-3 (show LTBP2 ELISA Kits) play an important regulatory role in TGF-beta (show TGFB1 ELISA Kits) activation and autocrine growth control in mesenchymal stem cells.
Marfan syndrome mice lacking LTBP-3 have improved survival, essentially no aneurysms, reduced disruption and fragmentation of medial elastic fibers, and decreased Smad2 (show SMAD2 ELISA Kits)/3 and Erk1/2 (show MAPK1/3 ELISA Kits) activation in their aortas.
These data indicate that LTBP-3 and -4 perform partially overlapping functions only in the lungs.
Bone abnormalities in latent TGF-[beta] binding protein (Ltbp)-3-null mice indicate a role for Ltbp-3 in modulating TGF-[beta] bioavailability
The secretion of LTBP-3 requires complexing of LTBP-3 with Cys33 of the TGF-beta (show TGFB1 ELISA Kits) propeptide.
Disruption of Ltbp-3 gene by homologous recombination in mice yields mutant animals displaying multiple skeletal abnormalities and retarded growth. Severe involution of thymus and spleen and reduction in CD4 (show CD4 ELISA Kits)/CD8 (show CD8A ELISA Kits) double positive T-cells in thymus.
Lack of Ltbp-3 results in decreased levels of TGF-beta (show TGFB1 ELISA Kits) in bone and cartilage, which leads to compromised osteoclast function and decreased bone turnover.
Loss of Ltbp-3 is associated with lung alveolar septation and developmental emphysema.
Embryos injected with an nkx2.5 morpholino exhibited SHF (show GJA1 ELISA Kits) phenotypes caused by compromised progenitor cell proliferation. Co-injecting low doses of nkx2.5 and ltbp3 morpholinos revealed a genetic interaction between these factors.
a requirement for ltbp3-TGF-beta signalling during zebrafish SHF development, a process that serves to enlarge the single ventricular chamber in this species
The protein encoded by this gene forms a complex with transforming growth factor beta (TGF-beta) proteins and may be involved in their subcellular localization. Activation of this complex requires removal of the encoded binding protein. This protein also may play a structural role in the extracellular matrix. Three transcript variants encoding different isoforms have been found for this gene.
latent TGF beta binding protein 3
, latent-transforming growth factor beta-binding protein 3
, latent transforming growth factor beta binding protein 3