C57BL/6JCya-Ggcxem1flox/Cya
Common Name:
Ggcx-flox
Product ID:
S-CKO-12023
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Ggcx-flox
Strain ID
CKOCMP-56316-Ggcx-B6J-VA
Gene Name
Product ID
S-CKO-12023
Gene Alias
--
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
6
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Ggcxem1flox/Cya mice (Catalog S-CKO-12023) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000065906
NCBI RefSeq
NM_019802
Target Region
Exon 3~4
Size of Effective Region
~1.4 kb
Detailed Document
Overview of Gene Research
Ggcx, also known as gamma-glutamyl carboxylase or vitamin K-dependent glutamyl carboxylase, is an enzyme that catalyzes the post-translational modification of specific glutamate residues in vitamin K-dependent proteins (VKDPs). This process, called gamma-carboxylation, is essential for activating VKDPs which are involved in multiple biological functions including blood coagulation, bone metabolism, vascular calcification, and cell proliferation [4].
Mutations in the GGCX gene are associated with multiple phenotypes. Vitamin K-dependent coagulation factor deficiency (VKCFD1) is a well-known phenotype, and patients may also have skin, eye, heart or bone manifestations [1]. Some GGCX variants that cause reduced gamma-carboxylation of Gla rich protein (GRP) are associated with skin laxity in VKCFD1 patients [2]. Different GGCX mutations show varying responses to vitamin K, determining the severity of the hemorrhagic phenotype in VKCFD1 patients, which helps in predicting the outcome and recommending appropriate treatments [3]. In a study on mice, the GgcxK325Q mutation did not affect the calcium homeostasis of the epididymis and male fertility [5]. Also, knocking out GGCX reduces the replication of Eurasian avian-like H1N1 swine influenza virus, as GGCX-mediated carboxylation modification of viral haemagglutinin (HA) contributes to the virus's receptor binding adaption [6].
In conclusion, Ggcx is crucial for the activation of VKDPs and thus for various biological functions. Research on Ggcx, especially through genetic models like the GgcxK325Q knock-in mice, has provided insights into its role in human diseases such as VKCFD1, and also in non-disease-related biological processes like male fertility and virus-host interactions.
References:
1. De Vilder, Eva Y G, Debacker, Jens, Vanakker, Olivier M. 2017. GGCX-Associated Phenotypes: An Overview in Search of Genotype-Phenotype Correlations. In International journal of molecular sciences, 18, . doi:10.3390/ijms18020240. https://pubmed.ncbi.nlm.nih.gov/28125048/
2. Ghosh, Suvoshree, Kraus, Katrin, Biswas, Arijit, Oldenburg, Johannes, Czogalla-Nitsche, Katrin J. 2021. GGCX variants leading to biallelic deficiency to γ-carboxylate GRP cause skin laxity in VKCFD1 patients. In Human mutation, 43, 42-55. doi:10.1002/humu.24300. https://pubmed.ncbi.nlm.nih.gov/34816548/
3. Ghosh, Suvoshree, Kraus, Katrin, Biswas, Arijit, Czogalla-Nitsche, Katrin J, Oldenburg, Johannes. 2021. GGCX mutations show different responses to vitamin K thereby determining the severity of the hemorrhagic phenotype in VKCFD1 patients. In Journal of thrombosis and haemostasis : JTH, 19, 1412-1424. doi:10.1111/jth.15238. https://pubmed.ncbi.nlm.nih.gov/33590680/
4. Shen, Guomin, Liu, Hongli, Shen, Yan, Xi, Shoumin. . [Partial research progress of GGCX pathogenic variation associated phenotypes]. In Zhonghua yi xue yi chuan xue za zhi = Zhonghua yixue yichuanxue zazhi = Chinese journal of medical genetics, 39, 646-650. doi:10.3760/cma.j.cn511374-20210430-00381. https://pubmed.ncbi.nlm.nih.gov/35773773/
5. Xiong, Mingxiang, Cheng, Pang, Liu, Bo, Gao, Ting, Li, Zhen. 2024. The GgcxK325Q Mutation Does Not Affect the Calcium Homeostasis of the Epididymis and Male Fertility in Mice. In Current issues in molecular biology, 46, 5052-5065. doi:10.3390/cimb46060303. https://pubmed.ncbi.nlm.nih.gov/38920975/
6. Zou, Jiahui, Jiang, Meijun, Xiao, Rong, Xie, Shengsong, Zhou, Hongbo. 2025. GGCX promotes Eurasian avian-like H1N1 swine influenza virus adaption to interspecies receptor binding. In Nature communications, 16, 670. doi:10.1038/s41467-025-55903-0. https://pubmed.ncbi.nlm.nih.gov/39809757/
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