C57BL/6JCya-Lamp2em1flox/Cya
Common Name:
Lamp2-flox
Product ID:
S-CKO-18593
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Lamp2-flox
Strain ID
CKOCMP-16784-Lamp2-B6J-VD
Gene Name
Product ID
S-CKO-18593
Gene Alias
CD107b; LGP-B; Lamp II; Lamp-2; Lamp-2a; Lamp-2b; Lamp-2c; Mac3
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
X
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Lamp2em1flox/Cya mice (Catalog S-CKO-18593) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000016678
NCBI RefSeq
NM_001017959
Target Region
Exon 3
Size of Effective Region
~1.1 kb
Detailed Document
Overview of Gene Research
Lamp2, short for lysosomal-associated membrane protein 2, is a well-recognized mediator of autolysosome maturation. It is involved in the autophagy-lysosomal protein degradation pathway, which is crucial for various biological processes. Mutations in the Lamp2 gene are associated with Danon disease, a rare X-linked dominant multisystemic disorder [2,5,6].
Genetic inactivation of Lamp2 in thymic stromal cells specifically impairs the development of CD4 T cells that have completed positive selection. This is due to defects in autophagy in Lamp2-deficient cortical thymic epithelial cells (cTECs), which are linked to alterations in MHC II processing and a reduction in CD4 TCR repertoire diversity [1].
In a LAMP2 knockdown cell model, LAMP2 deficiency led to a reduction of viral DNA replication and formation of infectious particles, and attenuated tau hyperphosphorylation and inhibition of Aβ secretion induced by HSV-1, suggesting its role in neurodegeneration-related processes [3].
In MDS/AML, LAMP2 deficiency is responsible for azacytidine resistance and hypersensitivity to lysosome and autophagy inhibitors, and low levels of LAMP2 expression in CD34 + blasts from patients correlate with poor overall survival [4].
In summary, Lamp2 plays essential roles in autophagy, T-cell development, neurodegeneration-related processes, and response to certain drugs in MDS/AML. Studies using gene knockout or knockdown models have been crucial in revealing these functions, especially in relation to Danon disease, neurodegenerative diseases, and hematological malignancies.
References：
1. Rodrigues, Pedro M, Sousa, Laura G, Perrod, Chiara, Saftig, Paul, Alves, Nuno L. 2022. LAMP2 regulates autophagy in the thymic epithelium and thymic stroma-dependent CD4 T cell development. In Autophagy, 19, 426-439. doi:10.1080/15548627.2022.2074105. https://pubmed.ncbi.nlm.nih.gov/35535798/
2. Shalata, Adel, Bar-Shai, Marina, Hadid, Yarin, Ben-Yosef, Tamar, Yaniv, Liat. 2023. Danon Disease: Entire LAMP2 Gene Deletion with Unusual Clinical Presentation-Case Report and Review of the Literature. In Genes, 14, . doi:10.3390/genes14081539. https://pubmed.ncbi.nlm.nih.gov/37628591/
3. Kristen, Henrike, Sastre, Isabel, Aljama, Sara, Bullido, Maria J, Aldudo, Jesus. 2021. LAMP2 deficiency attenuates the neurodegeneration markers induced by HSV-1 infection. In Neurochemistry international, 146, 105032. doi:10.1016/j.neuint.2021.105032. https://pubmed.ncbi.nlm.nih.gov/33781848/
4. Dubois, Alix, Furstoss, Nathan, Calleja, Anne, Auberger, Patrick, Robert, Guillaume. 2019. LAMP2 expression dictates azacytidine response and prognosis in MDS/AML. In Leukemia, 33, 1501-1513. doi:10.1038/s41375-018-0336-1. https://pubmed.ncbi.nlm.nih.gov/30607021/
5. Eskelinen, Eeva-Liisa. 2006. Roles of LAMP-1 and LAMP-2 in lysosome biogenesis and autophagy. In Molecular aspects of medicine, 27, 495-502. doi:. https://pubmed.ncbi.nlm.nih.gov/16973206/
6. Bottillo, Irene, Giordano, Carla, Cerbelli, Bruna, d'Amati, Giulia, Grammatico, Paola. 2016. A novel LAMP2 mutation associated with severe cardiac hypertrophy and microvascular remodeling in a female with Danon disease: a case report and literature review. In Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology, 25, 423-31. doi:10.1016/j.carpath.2016.07.005. https://pubmed.ncbi.nlm.nih.gov/27497751/
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