C57BL/6JCya-Abcc6em1flox/Cya
Common Name:
Abcc6-flox
Product ID:
S-CKO-10046
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Abcc6-flox
Strain ID
CKOCMP-27421-Abcc6-B6J-VA
Gene Name
Product ID
S-CKO-10046
Gene Alias
Abcc1b; DCC; Dyscalc1; Mrp6; dyscalc
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
7
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Abcc6em1flox/Cya mice (Catalog S-CKO-10046) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000002850
NCBI RefSeq
NM_018795
Target Region
Exon 5~6
Size of Effective Region
~2.3 kb
Detailed Document
Overview of Gene Research
ABCC6, encoding the trans-membrane protein ABCC6/MRP6, belongs to the adenosine triphosphate-binding cassette (ABC) gene subfamily C. It is primarily expressed in the liver and kidney. ABCC6 facilitates the cellular efflux of ATP, which is then converted into inorganic pyrophosphate (PPi) and adenosine, and is crucial for maintaining mineralization homeostasis as PPi is an inhibitor of calcification [1,2,3,4,6].
ABCC6 deficiency in mice underlies an inducible dystrophic cardiac calcification phenotype (DCC). Loss-of-function mutations in ABCC6 cause pseudoxanthoma elasticum (PXE), a multi-organ disease affecting dermal, ocular, and cardiovascular tissues, and also some cases of generalized arterial calcification of infancy (GACI). ABCC6 deficiency also promotes dyslipidemia and atherosclerosis in Ldlr-/-mice, with decreased HDL cholesterol and induced atherosclerosis [1,5].
In conclusion, ABCC6 is essential for maintaining normal mineralization and lipid metabolism. Mouse models with ABCC6 deficiency have been instrumental in revealing its role in ectopic calcification-related diseases like PXE and GACI, as well as in dyslipidemia and atherosclerosis, contributing to our understanding of the pathophysiology of these conditions [1,5].
References：
1. Shimada, Briana K, Pomozi, Viola, Zoll, Janna, Martin, Ludovic, Le Saux, Olivier. 2021. ABCC6, Pyrophosphate and Ectopic Calcification: Therapeutic Solutions. In International journal of molecular sciences, 22, . doi:10.3390/ijms22094555. https://pubmed.ncbi.nlm.nih.gov/33925341/
2. Favre, Guillaume, Laurain, Audrey, Aranyi, Tamas, Martin, Ludovic, Lefthériotis, Georges. 2017. The ABCC6 Transporter: A New Player in Biomineralization. In International journal of molecular sciences, 18, . doi:10.3390/ijms18091941. https://pubmed.ncbi.nlm.nih.gov/28891970/
3. Bergen, Arthur A B, Plomp, Astrid S, Hu, Xiaofeng, de Jong, Paulus T V M, Gorgels, Theo G M F. 2006. ABCC6 and pseudoxanthoma elasticum. In Pflugers Archiv : European journal of physiology, 453, 685-91. doi:. https://pubmed.ncbi.nlm.nih.gov/16604369/
4. Moitra, Karobi, Garcia, Sonia, Jaldin, Michelle, Terry, Sharon, Dean, Michael. 2017. ABCC6 and Pseudoxanthoma Elasticum: The Face of a Rare Disease from Genetics to Advocacy. In International journal of molecular sciences, 18, . doi:10.3390/ijms18071488. https://pubmed.ncbi.nlm.nih.gov/28696355/
5. Brampton, Christopher, Pomozi, Viola, Chen, Li-Hsieh, Martin, Ludovic, Le Saux, Olivier. 2021. ABCC6 deficiency promotes dyslipidemia and atherosclerosis. In Scientific reports, 11, 3881. doi:10.1038/s41598-021-82966-y. https://pubmed.ncbi.nlm.nih.gov/33594095/
6. Verschuere, Shana, Van Gils, Matthias, Nollet, Lukas, Vanakker, Olivier M. 2020. From membrane to mineralization: the curious case of the ABCC6 transporter. In FEBS letters, 594, 4109-4133. doi:10.1002/1873-3468.13981. https://pubmed.ncbi.nlm.nih.gov/33131056/
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