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C57BL/6JCya-Auts2em1flox/Cya
Common Name:
Auts2-flox
Product ID:
S-CKO-10369
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Price:
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Basic Information
Strain Name
Auts2-flox
Strain ID
CKOCMP-319974-Auts2-B6J-VA
Gene Name
Auts2
Product ID
S-CKO-10369
Gene Alias
2700063G02Rik; A730011F23Rik; D830032G16Rik
Background
C57BL/6JCya
NCBI ID
319974
Modification
Conditional knockout
Chromosome
5
Phenotype
MGI:1919847
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-Auts2em1flox/Cya mice (Catalog S-CKO-10369) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000161226
NCBI RefSeq
NM_001363480
Target Region
Exon 6
Size of Effective Region
~1.8 kb
Detailed Document
Click here to download >>
Overview of Gene Research
AUTS2, also known as Autism susceptibility candidate 2, is a crucial gene associated with a wide range of neuropsychological disorders, such as autism spectrum disorders (ASD), intellectual disability (ID), schizophrenia, and epilepsy [2]. It is involved in multiple neurodevelopmental processes. In cell nuclei, it acts as a key transcriptional regulator, while in the cytoplasm, it participates in cerebral corticogenesis, including neuronal migration and neuritogenesis, through controlling cytoskeletal rearrangements. Postnatally, it regulates the number of excitatory synapses to maintain the balance between excitation and inhibition in neural circuits [2]. Genetic models like zebrafish and mice are valuable tools for studying AUTS2 [1,3].

In mouse models, excision of different Auts2 exons led to distinct phenotypes. For example, excision of exon 15 caused breathing abnormalities and neonatal lethality when inactivated throughout the developing brain. Limited inactivation in the cerebral cortex resulted in cortical structure and function abnormalities, including dentate gyrus hypoplasia and abnormal EEG spiking activity [5]. Auts2 -cKO mice, with deletion specifically in Exm1 -positive neurons, exhibited ASD-like behaviors, reduced brain glucose uptake, inhibited pentose phosphate pathway, signs of oxidative stress, and disrupted mitochondrial homeostasis [4]. In zebrafish, auts2a morphants displayed microcephaly, which could be rescued by either the full-length or the C-terminal isoform of AUTS2 [1,3].

In conclusion, AUTS2 plays essential roles in neurodevelopment, regulating processes like neuronal migration, neuritogenesis, and RNA metabolism. Mouse models, especially KO and CKO mouse models, have significantly contributed to understanding its role in diseases such as ASD, intellectual disability, and related neurological disorders. These models help reveal the molecular mechanisms underlying AUTS2 -associated pathologies, providing insights into potential therapeutic strategies for these conditions.

References:
1. Biel, Alecia, Castanza, Anthony S, Rutherford, Ryan, Hester, Mark E, Hevner, Robert F. 2022. AUTS2 Syndrome: Molecular Mechanisms and Model Systems. In Frontiers in molecular neuroscience, 15, 858582. doi:10.3389/fnmol.2022.858582. https://pubmed.ncbi.nlm.nih.gov/35431798/
2. Hori, Kei, Shimaoka, Kazumi, Hoshino, Mikio. 2021. AUTS2 Gene: Keys to Understanding the Pathogenesis of Neurodevelopmental Disorders. In Cells, 11, . doi:10.3390/cells11010011. https://pubmed.ncbi.nlm.nih.gov/35011572/
3. Beunders, Gea, Voorhoeve, Els, Golzio, Christelle, Groffen, Alexander J, Sistermans, Erik A. 2013. Exonic deletions in AUTS2 cause a syndromic form of intellectual disability and suggest a critical role for the C terminus. In American journal of human genetics, 92, 210-20. doi:10.1016/j.ajhg.2012.12.011. https://pubmed.ncbi.nlm.nih.gov/23332918/
4. Liu, Min, Chen, Yimeng, Sun, Miao, Ma, Yulong, Wang, Guyan. 2022. Auts2 regulated autism-like behavior, glucose metabolism and oxidative stress in mice. In Experimental neurology, 361, 114298. doi:10.1016/j.expneurol.2022.114298. https://pubmed.ncbi.nlm.nih.gov/36525998/
5. Castanza, Anthony S, Ramirez, Sanja, Tripathi, Prem P, Ramirez, Jan-Marino, Hevner, Robert F. . AUTS2 Regulates RNA Metabolism and Dentate Gyrus Development in Mice. In Cerebral cortex (New York, N.Y. : 1991), 31, 4808-4824. doi:10.1093/cercor/bhab124. https://pubmed.ncbi.nlm.nih.gov/34013328/
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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