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C57BL/6NCya-Adss1em1flox/Cya
Common Name:
Adss1-flox
Product ID:
S-CKO-01087
Background:
C57BL/6NCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Adss1-flox
Strain ID
CKOCMP-11565-Adss1-B6N-VA
Gene Name
Adss1
Product ID
S-CKO-01087
Gene Alias
Adss; Adssl1
Background
C57BL/6NCya
NCBI ID
11565
Modification
Conditional knockout
Chromosome
12
Phenotype
MGI:87947
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/6NCya-Adss1em1flox/Cya mice (Catalog S-CKO-01087) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000021726
NCBI RefSeq
NM_007421
Target Region
Exon 6~9
Size of Effective Region
~1.9 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Adss1, encoding adenylosuccinate synthetase 1, is a key component in adenine nucleotide biosynthesis. It is one of the enzymes in the purine nucleotide cycle, which is muscle-specific [3]. Adenylosuccinate synthetase (ADSS) catalyzes a key step in AMP synthesis, and Adss1 is one of its isozymes in vertebrates [1].

In rodent models, Adss1 is up-regulated in surgically induced cardiac hypertrophy [2]. In an in vitro hypertrophy system of rat neonatal cardiomyocytes treated with angiotensin II, a signaling cascade activates the Adss1 gene, and this induction is blocked by cyclosporin A, suggesting calcineurin's involvement. The induction requires an NFAT binding site upstream of the Adss1 transcription start site [2]. Additionally, in cultured cardiomyocytes, electrical stimulation activates the NFAT3 and GATA4 pathways, leading to Adss1 gene up-regulation [5]. Mutations in Adss1 can cause ADSS1 myopathy, an autosomal recessive muscle disease complicated by respiratory muscle weakness, cardiomyopathy, and limb muscle weakness [4].

In conclusion, Adss1 is crucial for adenine nucleotide biosynthesis, especially in muscle-related metabolic cycles. Research on rodent models and in vitro cell systems has revealed its role in cardiac hypertrophy and muscle-related diseases. Understanding Adss1's function through these models provides insights into the mechanisms of cardiac and muscle-associated disorders, which may contribute to the development of related treatments.

References:
1. Li, Xinyun, Zhu, Zhengmao, Mo, Delin, Zhao, Shuhong, Li, Kui. 2007. Comparative molecular characterization of ADSS1 and ADSS2 genes in pig (Sus scrofa). In Comparative biochemistry and physiology. Part B, Biochemistry & molecular biology, 147, 271-7. doi:. https://pubmed.ncbi.nlm.nih.gov/17347008/
2. Wen, H Y, Xia, Y, Young, M E, Taegtmeyer, H, Kellems, R E. . The adenylosuccinate synthetase-1 gene is activated in the hypertrophied heart. In Journal of cellular and molecular medicine, 6, 235-43. doi:. https://pubmed.ncbi.nlm.nih.gov/12169208/
3. Lewis, A L, Guicherit, O M, Datta, S K, Hanten, G R, Kellems, R E. . Structure and expression of the murine muscle adenylosuccinate synthetase gene. In The Journal of biological chemistry, 271, 22647-56. doi:. https://pubmed.ncbi.nlm.nih.gov/8798436/
4. Hama, Yuka, Sano, Terunori, Oya, Yasushi, Takahashi, Yuji, Takao, Masaki. 2025. Two Brothers With ADSS1 Myopathy: A Report of Clinical, Radiological, and Autopsy Findings. In Neuropathology : official journal of the Japanese Society of Neuropathology, , . doi:10.1111/neup.70008. https://pubmed.ncbi.nlm.nih.gov/40302423/
5. Xia, Y, McMillin, J B, Lewis, A, Williams, R S, Kellems, R E. . Electrical stimulation of neonatal cardiac myocytes activates the NFAT3 and GATA4 pathways and up-regulates the adenylosuccinate synthetase 1 gene. In The Journal of biological chemistry, 275, 1855-63. doi:. https://pubmed.ncbi.nlm.nih.gov/10636885/
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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