C57BL/6JCya-Glb1em1/Cya
Common Name
Glb1-KO
Product ID
S-KO-16926
Backgroud
C57BL/6JCya
Strain ID
KOCMP-12091-Glb1-B6J-VA
Status
When using this mouse strain in a publication, please cite “Glb1-KO Mouse (Catalog S-KO-16926) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
The standard delivery applies for a guaranteed minimum of three heterozygous carriers. Breeding services for homozygous carriers and/or specified sex are available.
Basic Information
Strain Name
Glb1-KO
Strain ID
KOCMP-12091-Glb1-B6J-VA
Gene Name
Product ID
S-KO-16926
Gene Alias
Bge, Bgl, Bgs, Bgt, Bgl-e, Bgl-s, Bgl-t, C130097A14Rik
Background
C57BL/6JCya
NCBI ID
Modification
Conventional knockout
Chromosome
Chr 9
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Number
ENSMUST00000063042
NCBI RefSeq
NM_009752
Target Region
Exon 3
Size of Effective Region
~0.9 kb
Overview of Gene Research
Glb1, encoding lysosomal β-galactosidase, is of great biological significance. Lysosomal β-galactosidase is involved in the degradation of β-linked galactose-containing glycoconjugates in the lysosomal pathway [2,3]. Mutations in Glb1 are associated with GM1 gangliosidosis, a lysosomal storage disorder [3]. Mouse models are valuable for studying Glb1's functions.
A Glb1 knockout mouse model was generated using CRISPR/Cas9 genome editing. Glb1-/-mice have small deletions in exons 2 and 6, creating a null allele. These mice have a lifespan of approximately 50 weeks, with female mice dying six weeks earlier than males. They exhibit progressive gait abnormalities, loss of motor skills, and cerebellar atrophy, similar to type II GM1 gangliosidosis patients. Additionally, Glb1-/-mice show increased levels of a novel pentasaccharide biomarker in urine and plasma, as well as accumulation of glycosphingolipids in the brain [1].
In conclusion, Glb1 is essential for the normal degradation of specific glycoconjugates through its encoded lysosomal β-galactosidase. The Glb1 knockout mouse model closely mimics the features of type II GM1 gangliosidosis, facilitating the understanding of the disease mechanism and guiding the development of new therapies for this disorder [1,3].
References:
1. Nicoli, Elena-Raluca, Huebecker, Mylene, Han, Sangwoo T, Platt, Frances M, Tifft, Cynthia J. 2023. Glb1 knockout mouse model shares natural history with type II GM1 gangliosidosis patients. In Molecular genetics and metabolism, 138, 107508. doi:10.1016/j.ymgme.2023.107508. https://pubmed.ncbi.nlm.nih.gov/36709532/
2. Lee, Bo Yun, Han, Jung A, Im, Jun Sub, DiMaio, Daniel, Hwang, Eun Seong. . Senescence-associated beta-galactosidase is lysosomal beta-galactosidase. In Aging cell, 5, 187-95. doi:. https://pubmed.ncbi.nlm.nih.gov/16626397/
3. Nicoli, Elena-Raluca, Annunziata, Ida, d'Azzo, Alessandra, Tifft, Cynthia J, Stepien, Karolina M. 2021. GM1 Gangliosidosis-A Mini-Review. In Frontiers in genetics, 12, 734878. doi:10.3389/fgene.2021.734878. https://pubmed.ncbi.nlm.nih.gov/34539759/
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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