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Mouse Anti-LTBP1 Recombinant Antibody (CBYJL-2147) (CBMAB-L2321-YJ)

Provided herein is a Mouse monoclonal antibody, which binds to Latent Transforming Growth Factor Beta Binding Protein 1 (LTBP1). The antibody can be used for immunoassay techniques, such as WB.
See all LTBP1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBYJL-2147
Antibody Isotype
IgG1
Application
WB

Basic Information

Immunogen
Recombinant protein corresponding to human LTBP-1.
Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4
Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
latent transforming growth factor beta binding protein 1
Introduction
LTBP1 belongs to the family of latent TGF-beta binding proteins (LTBPs). The secretion and activation of TGF-betas is regulated by their association with latency-associated proteins and with latent TGF-beta binding proteins. LTBP1 targets latent complexes of transforming growth factor beta to the extracellular matrix, where the latent cytokine is subsequently activated by several different mechanisms. Alternatively spliced transcript variants encoding different isoforms have been identified.
Entrez Gene ID
4052
UniProt ID
Q14766
Alternative Names
latent-transforming growth factor beta-binding protein 1; LTBP-1; TGF-beta1-BP-1; transforming growth factor beta-1-binding protein 1
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 (PubMed:2022183, PubMed:8617200, PubMed:8939931).
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 (PubMed:8617200, PubMed:8939931, PubMed:15184403).
Outcompeted by LRRC32/GARP for binding to LAP regulatory chain of TGF-beta (PubMed:22278742).
Biological Process
Regulation of transforming growth factor beta activationManual Assertion Based On ExperimentIDA:UniProtKB
Sequestering of TGFbeta in extracellular matrixManual Assertion Based On ExperimentIDA:UniProtKB
Cellular Location
Secreted
Secreted, extracellular space, extracellular matrix
Involvement in disease
Cutis laxa, autosomal recessive, 2E (ARCL2E):
A form of cutis laxa, a disorder characterized by an excessive congenital skin wrinkling, a large fontanelle with delayed closure, a typical facial appearance with downslanting palpebral fissures, and a general connective tissue weakness. ARCL2E patients present with cutis laxa, inguinal hernia, craniofacial dysmorphology, variable heart defects, and prominent skeletal features including craniosynostosis, short stature, brachydactyly, and syndactyly.
PTM
Contains hydroxylated asparagine residues.
Isoform Short N-terminus is blocked.
Two intrachain disulfide bonds from the TB3 domain are rearranged upon TGFB1 binding, and form interchain bonds with TGFB1 propeptide, anchoring it to the extracellular matrix.
O-glycosylated on serine residues by POGLUT2 and POGLUT3.

Li, C., Zhang, L., Bu, X., Wang, J., Li, L., & Yang, Z. (2022). Circ-LTBP1 is involved in doxorubicin-induced intracellular toxicity in cardiomyocytes via miR-107/ADCY1 signal. Molecular and Cellular Biochemistry, 477(4), 1127-1138.

Przyklenk, M., Georgieva, V. S., Metzen, F., Mostert, S., Kobbe, B., Callewaert, B., ... & Schiavinato, A. (2022). LTBP1 promotes fibrillin incorporation into the extracellular matrix. Matrix Biology, 110, 60-75.

Williamson, D. B., Sohn, C. J., Ito, A., & Haltiwanger, R. S. (2021). POGLUT2 and POGLUT3 O-glucosylate multiple EGF repeats in fibrillin-1,-2, and LTBP1 and promote secretion of fibrillin-1. Journal of Biological Chemistry, 297(3).

Lin, R., Li, X., Wu, S., Qian, S., Hou, H., Dong, M., ... & Zhang, M. (2021). Suppression of latent transforming growth factor-β (TGF-β)-binding protein 1 (LTBP1) inhibits natural killer/T cell lymphoma progression by inactivating the TGF-β/Smad and p38MAPK pathways. Experimental Cell Research, 407(1), 112790.

Pottie, L., Adamo, C. S., Beyens, A., Lütke, S., Tapaneeyaphan, P., De Clercq, A., ... & Callewaert, B. (2021). Bi-allelic premature truncating variants in LTBP1 cause cutis laxa syndrome. The American Journal of Human Genetics, 108(6), 1095-1114.

Zhao, Q., Zheng, K., Ma, C., Li, J., Zhuo, L., Huang, W., ... & Jiang, Y. (2020). PTPS facilitates compartmentalized LTBP1 S-nitrosylation and promotes tumor growth under hypoxia. Molecular cell, 77(1), 95-107.

Cai, R., Wang, P., Zhao, X., Lu, X., Deng, R., Wang, X., ... & Lin, J. (2020). LTBP1 promotes esophageal squamous cell carcinoma progression through epithelial-mesenchymal transition and cancer-associated fibroblasts transformation. Journal of Translational Medicine, 18(1), 1-13.

Fu, X., Zhang, P., Song, H., Wu, C., Li, S., Li, S., & Yan, C. (2020). LTBP1 plays a potential bridge between depressive disorder and glioblastoma. Journal of translational medicine, 18(1), 1-21.

Qian, H., Li, J., Zou, L., Ji, C., Li, H., Zheng, Y., ... & Zhang, Y. (2020). LTBP1-ALK: A novel fusion identified in malignant pleural effusions from a patient with advanced lung adenocarcinoma. Lung Cancer, 144, 93-97.

Mami, S., Ghaffarpour, S., Faghihzadeh, S., & Ghazanfari, T. (2019). Evaluation of the LTBP1 and Smad6 genes expression in lung tissue of Sulfur mustard-exposed individuals with long-term pulmonary complications. Iranian Journal of Allergy, Asthma and Immunology, 473-478.

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For research use only. Not intended for any clinical use.

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