C57BL/6JCya-Gckem1flox/Cya
Common Name:
Gck-flox
Product ID:
S-CKO-00353
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
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Basic Information
Strain Name
Gck-flox
Strain ID
CKOCMP-103988-Gck-B6J-VC
Gene Name
Product ID
S-CKO-00353
Gene Alias
GLK; Gk; Gls006; HK4; HKIV; HXKP; Hlb62; MODY2
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
11
Phenotype
Document
Application
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Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Gckem1flox/Cya mice (Catalog S-CKO-00353) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000102920
NCBI RefSeq
NM_010292
Target Region
Exon 9~10
Size of Effective Region
~2.7 kb
Detailed Document
Overview of Gene Research
Gck, short for glucokinase, is a key enzyme in glucose metabolism. It has a central role in glucose sensing and insulin secretion in pancreatic β -cells, as well as glycogen synthesis in the liver, thus being crucial for maintaining glucose homeostasis [3].
Mutations in the Gck gene are associated with various monogenic diabetes disorders. Heterozygous inactivating mutations cause GCK-MODY, a form of Maturity Onset Diabetes of the Young, characterized by fasting hyperglycemia, low risk for chronic vascular complications, and often misdiagnosed as type 1 or type 2 diabetes [1]. Homozygous inactivating mutations lead to permanent neonatal diabetes mellitus (PNDM) [3,4,6]. On the other hand, heterozygous activating mutations result in hyperinsulinemic hypoglycemia (HH) [3,4,6]. The penetrance of pathogenic Gck variants is similar across different cohorts (89%-97%) [2]. In addition, the glucokinase activator dorzagliatin was effective and safe in a patient with GCK-MODY, providing potential for precise treatment [5].
In conclusion, Gck is essential for glucose metabolism, regulating insulin secretion and glycogen synthesis. The study of Gck-related mutations through genetic models helps to understand the pathogenesis of monogenic diabetes disorders such as GCK-MODY, PNDM, and HH, which may provide insights for developing targeted therapeutic interventions [3].
References:
1. Hulín, J, Škopková, M, Valkovičová, T, Gašperíková, D, Staník, J. 2020. Clinical implications of the glucokinase impaired function - GCK MODY today. In Physiological research, 69, 995-1011. doi:. https://pubmed.ncbi.nlm.nih.gov/33129248/
2. Mirshahi, Uyenlinh L, Colclough, Kevin, Wright, Caroline F, Weedon, Michael N, Patel, Kashyap A. 2022. Reduced penetrance of MODY-associated HNF1A/HNF4A variants but not GCK variants in clinically unselected cohorts. In American journal of human genetics, 109, 2018-2028. doi:10.1016/j.ajhg.2022.09.014. https://pubmed.ncbi.nlm.nih.gov/36257325/
3. Abu Aqel, Yasmin, Alnesf, Aldana, Aigha, Idil I, Teo, Adrian, Abdelalim, Essam M. 2024. Glucokinase (GCK) in diabetes: from molecular mechanisms to disease pathogenesis. In Cellular & molecular biology letters, 29, 120. doi:10.1186/s11658-024-00640-3. https://pubmed.ncbi.nlm.nih.gov/39245718/
4. Osbak, Kara K, Colclough, Kevin, Saint-Martin, Cecile, Ellard, Sian, Gloyn, Anna L. . Update on mutations in glucokinase (GCK), which cause maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemic hypoglycemia. In Human mutation, 30, 1512-26. doi:10.1002/humu.21110. https://pubmed.ncbi.nlm.nih.gov/19790256/
5. Zhao, Yilin, Ma, Yumin, Ba, Tianhao, Ren, Qian, Ji, Linong. . Hypoglycemic Response to Dorzagliatin in a Patient With GCK-MODY. In Diabetes care, 47, 1140-1142. doi:10.2337/dc23-2417. https://pubmed.ncbi.nlm.nih.gov/38691834/
6. Gloyn, Anna L. . Glucokinase (GCK) mutations in hyper- and hypoglycemia: maturity-onset diabetes of the young, permanent neonatal diabetes, and hyperinsulinemia of infancy. In Human mutation, 22, 353-62. doi:. https://pubmed.ncbi.nlm.nih.gov/14517946/
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