C57BL/6JCya-Sftpcem1flox/Cya
Common Name:
Sftpc-flox
Product ID:
S-CKO-18636
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Sftpc-flox
Strain ID
CKOCMP-20389-Sftpc-B6J-VB
Gene Name
Product ID
S-CKO-18636
Gene Alias
Bricd6; SP-C; SP5; SPC; Sftp-2; Sftp2; pro-SpC
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
14
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Sftpcem1flox/Cya mice (Catalog S-CKO-18636) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000022692
NCBI RefSeq
NM_011359
Target Region
Exon 2~4
Size of Effective Region
~1.4 kb
Detailed Document
Overview of Gene Research
Sftpc, which encodes surfactant protein C, is crucial for pulmonary development and homeostasis. Surfactant protein C is produced by alveolar type 2 (AT2) cells and is essential for maintaining normal lung function, such as reducing surface tension in the alveoli. Dysfunction in related pathways may lead to various lung-related issues [1,2,3,4,5,7]. Genetic models, like gene-edited mouse models, are valuable for studying Sftpc's function.
In a study using patient-specific induced pluripotent stem cells (iPSCs) carrying an SFTPC I73T mutation, mutant iAEC2s (differentiated from iPSCs) accumulated misprocessed and mistrafficked pro-SFTPC protein. This led to diminished AEC2 progenitor capacity, perturbed proteostasis, altered bioenergetic programs, metabolic reprogramming, and NF-κB pathway activation [1].
In another study, Sftpc-knockout (KO) mouse models were used to explore its role in neurodevelopmental disorders. Compound heterozygous variants of SFTPC in children with NDDs led to reduced Sftpc protein levels. Sftpc-knock-in (Sftpc-KI) mice carrying defective alleles showed reduced Sftpc expression in the lung and blood, along with impaired neurobehavioral performance and dysregulated proteins associated with neuroinflammation in the brain [6].
In conclusion, Sftpc is vital for normal lung function, especially in maintaining alveolar homeostasis. Model-based research, such as KO/CKO mouse models, has revealed its role in not only lung-related diseases like interstitial lung disease but also in unexpected areas like neurodevelopmental disorders. Understanding Sftpc's function through these models provides insights into disease mechanisms and potential therapeutic targets.
References:
1. Alysandratos, Konstantinos-Dionysios, Russo, Scott J, Petcherski, Anton, Beers, Michael F, Kotton, Darrell N. . Patient-specific iPSCs carrying an SFTPC mutation reveal the intrinsic alveolar epithelial dysfunction at the inception of interstitial lung disease. In Cell reports, 36, 109636. doi:10.1016/j.celrep.2021.109636. https://pubmed.ncbi.nlm.nih.gov/34469722/
2. Abdel Megeid, Azza K, Refeat, Miral M, Ashaat, Engy A, El Ruby, Mona O, Amr, Khalda S. 2022. Correlating SFTPC gene variants to interstitial lung disease in Egyptian children. In Journal, genetic engineering & biotechnology, 20, 117. doi:10.1186/s43141-022-00399-0. https://pubmed.ncbi.nlm.nih.gov/35939165/
3. Katzen, Jeremy, Beers, Michael F. . Contributions of alveolar epithelial cell quality control to pulmonary fibrosis. In The Journal of clinical investigation, 130, 5088-5099. doi:10.1172/JCI139519. https://pubmed.ncbi.nlm.nih.gov/32870817/
4. Barkauskas, Christina E, Cronce, Michael J, Rackley, Craig R, Noble, Paul W, Hogan, Brigid L M. 2013. Type 2 alveolar cells are stem cells in adult lung. In The Journal of clinical investigation, 123, 3025-36. doi:10.1172/JCI68782. https://pubmed.ncbi.nlm.nih.gov/23921127/
5. Li, Ning, Liu, Bohao, Xiong, Rui, Wang, Bo, Geng, Qing. 2023. HDAC3 deficiency protects against acute lung injury by maintaining epithelial barrier integrity through preserving mitochondrial quality control. In Redox biology, 63, 102746. doi:10.1016/j.redox.2023.102746. https://pubmed.ncbi.nlm.nih.gov/37244125/
6. Dong, Haipeng, Zang, Congwen, Liu, Lili, Hu, Hao, Li, Na. 2025. Lung-specific SFTPC mutations lead to neurodevelopmental disorders with neuroinflammation. In Biochemical and biophysical research communications, 753, 151479. doi:10.1016/j.bbrc.2025.151479. https://pubmed.ncbi.nlm.nih.gov/39965266/
7. Diesler, Rémi, Legendre, Marie, Si-Mohamed, Salim, Borie, Raphaël, Cottin, Vincent. 2024. Similarities and differences of interstitial lung disease associated with pathogenic variants in SFTPC and ABCA3 in adults. In Respirology (Carlton, Vic.), 29, 312-323. doi:10.1111/resp.14667. https://pubmed.ncbi.nlm.nih.gov/38345107/
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