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C57BL/6JCya-Ltbp1em1flox/Cya
Common Name:
Ltbp1-flox
Product ID:
S-CKO-09732
Background:
C57BL/6JCya
Product Type
Age
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Basic Information
Strain Name
Ltbp1-flox
Strain ID
CKOCMP-268977-Ltbp1-B6J-VA
Gene Name
Ltbp1
Product ID
S-CKO-09732
Gene Alias
9430031G15Rik; 9830146M04; Ltbp-1; Ltbp1L; TGF-beta1-BP-1; Tgfb; b2b1000Clo
Background
C57BL/6JCya
NCBI ID
268977
Modification
Conditional knockout
Chromosome
17
Phenotype
MGI:109151
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ltbp1em1flox/Cya mice (Catalog S-CKO-09732) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000001927
NCBI RefSeq
NM_019919
Target Region
Exon 7
Size of Effective Region
~1.1 kb
Detailed Document
Click here to download >>
Overview of Gene Research
LTBP1, the latent transforming growth factor β binding protein 1, is a large extracellular matrix protein. It is an associated ligand of fibrillin-microfibrils and is well-known for its role in targeting and sequestering TGFβ growth factors within the extracellular matrix, regulating their bioavailability. This function is crucial for maintaining the homeostasis of connective tissues [1].

In in vitro studies, LTBP1 coordinates the incorporation of fibrillin-1 and-2 into the extracellular matrix, and this function is differentially exerted by its short and long isoforms, uncovering a TGFβ-independent role potentially related to connective tissue disorders [1]. In human patients, bi-allelic premature truncating variants in LTBP1 cause cutis laxa syndrome, with affected individuals showing connective tissue features, craniofacial dysmorphology, heart defects, and skeletal features. In vitro studies on proband-derived dermal fibroblasts and in vivo validation in zebrafish carrying ltbp1 mutations indicate that LTBP1 function is crucial for skin and bone ECM assembly and homeostasis [3]. In esophageal squamous cell carcinoma (ESCC), down-regulation of LTBP1 inhibits the invasion, migration, and metastatic abilities of cancer cells, and also reduces mesenchymal phenotypes and inhibits TGFβ-induced epithelial-mesenchymal transition (EMT). It also enhances ESCC cells' sensitivity to 5-FU treatment and attenuates cancer-associated fibroblasts (CAFs) transformation, suggesting an oncogenic role of LTBP1 in ESCC progression [2].

In conclusion, LTBP1 plays essential roles in extracellular matrix formation and connective tissue homeostasis. Its functions in diseases like cutis laxa syndrome and ESCC have been revealed through in vitro and in vivo studies including those using zebrafish models. Understanding LTBP1's functions can potentially provide new insights for treating connective tissue-related disorders and certain cancers.

References:
1. Przyklenk, Matthias, Georgieva, Veronika S, Metzen, Fabian, Koch, Manuel, Schiavinato, Alvise. 2022. LTBP1 promotes fibrillin incorporation into the extracellular matrix. In Matrix biology : journal of the International Society for Matrix Biology, 110, 60-75. doi:10.1016/j.matbio.2022.04.004. https://pubmed.ncbi.nlm.nih.gov/35452817/
2. Cai, Rui, Wang, Ping, Zhao, Xin, Hong, Chang, Lin, Jie. 2020. LTBP1 promotes esophageal squamous cell carcinoma progression through epithelial-mesenchymal transition and cancer-associated fibroblasts transformation. In Journal of translational medicine, 18, 139. doi:10.1186/s12967-020-02310-2. https://pubmed.ncbi.nlm.nih.gov/32216815/
3. Pottie, Lore, Adamo, Christin S, Beyens, Aude, Sengle, Gerhard, Callewaert, Bert. 2021. Bi-allelic premature truncating variants in LTBP1 cause cutis laxa syndrome. In American journal of human genetics, 108, 1095-1114. doi:10.1016/j.ajhg.2021.04.016. https://pubmed.ncbi.nlm.nih.gov/33991472/
Quality Control Standard
Sperm Test

Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.

Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.

Environmental Standards:SPF
Available Region:Global
Source:Cyagen
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