C57BL/6JCya-Extl2em1/Cya
Common Name:
Extl2-KO
Product ID:
S-KO-16420
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Extl2-KO
Strain ID
KOCMP-58193-Extl2-B6J-VB
Gene Name
Product ID
S-KO-16420
Gene Alias
3000001D04Rik
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
3
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Extl2em1/Cya mice (Catalog S-KO-16420) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000029575
NCBI RefSeq
NM_021388.4
Target Region
Exon 3
Size of Effective Region
~1.7 kb
Detailed Document
Overview of Gene Research
EXTL2, an N-acetylhexosaminyltransferase-encoding gene, is one of the three EXT-like genes in the human genome homologous to EXT1 and EXT2. It plays a role in glycosaminoglycan (GAG) biosynthesis. Specifically, it can transfer a GlcNAc residue to the phosphorylated tetrasaccharide linkage region by xylose kinase 1 (FAM20B), terminating GAG chain elongation [1,3]. This indicates its importance in regulating GAG biosynthesis and associated pathways [1,3]. Gene-knockout mouse models have been crucial in studying EXTL2's functions [1,3,4,5].
In EXTL2-knockout mice, GAG production is significantly higher than in wild-type mice [1,3]. Under carbon tetrachloride-induced liver failure, hepatocyte proliferation and liver regeneration are impaired in EXTL2-knockout mice due to reduced hepatocyte-growth-factor-mediated signaling [1,5]. In a model of chronic kidney disease, matrix mineralization in vascular smooth muscle cells of EXTL2-knockout mice is enhanced, as altered GAG biosynthesis affects bone-morphogenetic-protein signaling, promoting the differentiation of these cells into osteoblasts [1]. Also, in a demyelinating injury model, EXTL2-knockout mice had excessive CSPG deposition, exacerbated axonal damage, and increased microglia/macrophages in lesions [2]. Additionally, in the context of non-alcoholic steatohepatitis and hepatocarcinoma, GAGs produced without EXTL2 act as DAMPs, signaling via Toll-like 4 receptor and contributing to inflammation-driven tumor promotion [4].
In conclusion, EXTL2-mediated regulation of GAG biosynthesis is vital for maintaining tissue homeostasis under pathological conditions. Studies using EXTL2-knockout mouse models have revealed its role in various disease-related processes, including liver regeneration, aortic calcification, neuroinflammation, and liver tumorigenesis. Understanding EXTL2's functions provides insights into the mechanisms underlying these diseases and may offer potential therapeutic targets [1,2,4,5].
References:
1. Nadanaka, Satomi, Kitagawa, Hiroshi. 2013. EXTL2 controls liver regeneration and aortic calcification through xylose kinase-dependent regulation of glycosaminoglycan biosynthesis. In Matrix biology : journal of the International Society for Matrix Biology, 35, 18-24. doi:10.1016/j.matbio.2013.10.010. https://pubmed.ncbi.nlm.nih.gov/24176719/
2. Pu, Annie, Mishra, Manoj K, Dong, Yifei, Sawcer, Stephen, Yong, V Wee. 2020. The glycosyltransferase EXTL2 promotes proteoglycan deposition and injurious neuroinflammation following demyelination. In Journal of neuroinflammation, 17, 220. doi:10.1186/s12974-020-01895-1. https://pubmed.ncbi.nlm.nih.gov/32703234/
3. Nadanaka, Satomi, Zhou, Shaobo, Kagiyama, Shoji, Asano, Masahide, Kitagawa, Hiroshi. 2013. EXTL2, a member of the EXT family of tumor suppressors, controls glycosaminoglycan biosynthesis in a xylose kinase-dependent manner. In The Journal of biological chemistry, 288, 9321-33. doi:10.1074/jbc.M112.416909. https://pubmed.ncbi.nlm.nih.gov/23395820/
4. Nadanaka, Satomi, Hashiguchi, Taishi, Kitagawa, Hiroshi. 2020. Aberrant glycosaminoglycan biosynthesis by tumor suppressor EXTL2 deficiency promotes liver inflammation and tumorigenesis through Toll-like 4 receptor signaling. In FASEB journal : official publication of the Federation of American Societies for Experimental Biology, 34, 8385-8401. doi:10.1096/fj.201902076R. https://pubmed.ncbi.nlm.nih.gov/32347583/
5. Nadanaka, Satomi, Kagiyama, Shoji, Kitagawa, Hiroshi. . Roles of EXTL2, a member of the EXT family of tumour suppressors, in liver injury and regeneration processes. In The Biochemical journal, 454, 133-45. doi:10.1042/BJ20130323. https://pubmed.ncbi.nlm.nih.gov/23734945/
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