C57BL/6JCya-Alg13em1flox/Cya
Common Name
Alg13-flox
Product ID
S-CKO-13762
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-67574-Alg13-B6J-VA
When using this mouse strain in a publication, please cite “Alg13-flox Mouse (Catalog S-CKO-13762) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Basic Information
Strain Name
Alg13-flox
Strain ID
CKOCMP-67574-Alg13-B6J-VA
Gene Name
Product ID
S-CKO-13762
Gene Alias
2810046O15Rik, 4833435D08Rik, Glt28d1, MDS031
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr X
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000070801
NCBI RefSeq
NM_026247
Target Region
Exon 3~4
Size of Effective Region
~2.4 kb
Overview of Gene Research
ALG13, encoding a subunit of the uridine diphosphate-N-acetylglucosamine (UDP-GlcNAc) transferase enzyme, is crucial in the N-linked glycosylation pathway. This pathway attaches carbohydrate structures to asparagine residues in proteins within the endoplasmic reticulum, and N-glycosylated proteins are involved in processes like electrical gradients formation and neurotransmission [1].
In Alg13 knockout (KO) mouse models, KA-induced epileptic progressions were increased, including prolonged electrographic seizures, higher mortality rates, and more severe responses to epileptic seizures. The cortex and hippocampus of Alg13 KO mice showed hyperactive mTOR signaling pathways [2]. Also, about 20% of adult ALG13KO knockout mice display spontaneous seizures, with a marked decrease in gamma-aminobutyric acid A receptor (GABAAR)-mediated inhibitory synaptic transmission. ALG13 may influence the expression of GABAARα2 membrane and total protein by changing its transcription level [3].
In conclusion, ALG13 is essential for the N-linked glycosylation pathway. Studies using Alg13 KO mouse models have revealed its role in epilepsy, showing that ALG13 deficiency can increase seizure susceptibility and severity, potentially through the regulation of GABAAR function and mTOR signaling pathways [2,3].
References：
1. Gao, Peng, Chen, Haoran, Sun, Yangyang, Fan, Yuhan, Zhang, Jing. 2024. ALG13-Related Epilepsy: Current Insights and Future Research Directions. In Neurochemical research, 50, 60. doi:10.1007/s11064-024-04300-y. https://pubmed.ncbi.nlm.nih.gov/39673593/
2. Gao, Peng, Wang, Feng, Huo, Junming, Yu, Baoli, Sun, Tao. 2019. ALG13 Deficiency Associated with Increased Seizure Susceptibility and Severity. In Neuroscience, 409, 204-221. doi:10.1016/j.neuroscience.2019.03.009. https://pubmed.ncbi.nlm.nih.gov/30872163/
3. Huo, Junming, Ren, Shuanglai, Gao, Peng, Wang, Feng, Sun, Tao. 2020. ALG13 participates in epileptogenesis via regulation of GABAA receptors in mouse models. In Cell death discovery, 6, 87. doi:10.1038/s41420-020-00319-6. https://pubmed.ncbi.nlm.nih.gov/33014431/
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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