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The LTBP4 VTTT allele is associated with increased risk of dilated cardiomyopathy in European Americans. LTBP4 protein with the IAAM residues bound more latent TGFbeta (show TGFB1 ELISA Kits) compared to the LTBP4 VTTT protein.
Our results show that LTBP4 interacts with TGFBR2 (show TGFBR2 ELISA Kits) and stabilizes TGFbeta (show TGFB1 ELISA Kits) receptors by preventing their endocytosis and lysosomal degradation in a ligand-dependent and receptor kinase activity-dependent manner.
We show that corticosteroid treatment and the IAAM haplotype of the LTBP4 gene are significantly associated with prolonged ambulation in patients with Duchenne muscular dystrophy (show DMD ELISA Kits)
In the mdx (show DMD ELISA Kits) mouse model of Duchenne muscular dystrophy (show DMD ELISA Kits), the human LTBP4 transgene exacerbated muscular dystrophy symptoms and resulted in weaker muscles with an increased inflammatory infiltrate.
In Caucasians with Duchenne muscular dystrophy (show DMD ELISA Kits) and LTBP4 genotype there was a protective effect on age at loss of ambulation.
It recruits elastin (show ELN ELISA Kits) to microfibrils via fibulin-5 (show FBLN5 ELISA Kits).
LTBP4 haplotype influences age at loss of ambulation, and should be considered in the management of Duchenne muscular dystrophy (show DMD ELISA Kits) patients.
Latent transforming growth factor beta-binding protein 4 is downregulated in esophageal cancer via promoter methylation.
Data indicate mutations of FBLN4 (show FBLN4 ELISA Kits), FBLN5 (show FBLN5 ELISA Kits), and LTBP4 in 12 probands presenting with type 1 recessive cutis laxa.
the G1 and G3 domains of versican (show Vcan ELISA Kits) were upregulated and LTBP-4 was downregulated in breast cancer stroma
Ltbp-4L expression is essential for incorporation of fibulin-4 (show FBLN4 ELISA Kits) into the extracellular matrix.
These results are consistent with a role for TGFbeta2 in lung septation and for Ltbp4 in regulating fibulin-5 (show FBLN5 ELISA Kits) dependent elastic fiber assembly.
data suggest a unique function for LTBP-4 during elastic fibrogenesis, making it a potential therapeutic target for elastic fiber regeneration
The lack of LTBP4-mediated targeting in malignant mammary tumor tissues may lead to a possible modification of TGF-ss1 and BMP bioavailability and function.
These data indicate that LTBP-3 (show LTBP3 ELISA Kits) and -4 perform partially overlapping functions only in the lungs.
an insertion/deletion polymorphism of 36 bp in the coding region of the Ltbp4 was found in an animal model of muscular dystrophy
LTBP-4 has a role in targeting and activation of TGF-beta1 (show TGFB1 ELISA Kits) which regulates BMP-4 (show BMP4 ELISA Kits) signaling in mouse lung
Results indicate taht there are two separate functions of LTBP-4, as a regulator of elastic fiber assembly and of TGF-beta (show TGFB1 ELISA Kits) levels in lungs.
The protein encoded by this gene binds transforming growth factor beta (TGFB) as it is secreted and targeted to the extracellular matrix. TGFB is biologically latent after secretion and insertion into the extracellular matrix, and sheds TGFB and other proteins upon activation. Defects in this gene may be a cause of cutis laxa and severe pulmonary, gastrointestinal, and urinary abnormalities. Three transcript variants encoding different isoforms have been found for this gene.
latent transforming growth factor beta binding protein 4
, latent-transforming growth factor beta-binding protein 4-like
, latent transforming growth factor-beta binding protein 4L
, latent-transforming growth factor beta-binding protein 4