C57BL/6JCya-Osbpl2em1flox/Cya
Common Name:
Osbpl2-flox
Product ID:
S-CKO-18292
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
Quantity
Price:
Contact for Pricing
Basic Information
Strain Name
Osbpl2-flox
Strain ID
CKOCMP-228983-Osbpl2-B6J-VB
Gene Name
Product ID
S-CKO-18292
Gene Alias
C130070J12Rik; ORP-2; Orp2
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
2
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Osbpl2em1flox/Cya mice (Catalog S-CKO-18292) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000040668
NCBI RefSeq
NM_144500
Target Region
Exon 4
Size of Effective Region
~0.9 kb
Detailed Document
Overview of Gene Research
Osbpl2, also known as oxysterol-binding protein-related protein 2, is an important regulator in cellular lipid metabolism and transport [1]. It is involved in multiple cellular processes such as maintaining ciliogenesis by regulating the homeostasis of PI(4,5)P2 on the cilia membrane [2], and is associated with pathways like the Sonic Hedgehog (Shh) signaling pathway [2].
In gene-knockout models, Osbpl2-KO mice exhibited progressive hearing loss, abnormal cochlear development with defective cilia, and down-regulation of key molecules in the Shh signaling pathway [2]. Osbpl2-disrupted pigs showed progressive hearing loss, hypercholesterolaemia, and high-fat diet aggravated these phenotypes [1]. In HEI-OC1 cells, knockdown of Osbpl2 augmented cell death and apoptosis induced by H2O2, and inhibited the AKT/FOXG1 signaling pathway [3]. Deletion of Osbpl2 in auditory cells increased cholesterol biosynthesis and ROS production by inhibiting AMPK activity [4].
In conclusion, Osbpl2 is crucial for maintaining normal physiological functions related to lipid metabolism, cilia formation, and cell survival. The gene-knockout mouse and pig models have significantly contributed to understanding its role in diseases such as hearing loss, hypercholesterolaemia, and age-related hearing loss. These findings provide insights into the underlying mechanisms of these diseases and potential therapeutic targets.
References:
1. Yao, Jun, Zeng, Huasha, Zhang, Min, Cao, Xin, Dai, Yifan. 2019. OSBPL2-disrupted pigs recapitulate dual features of human hearing loss and hypercholesterolaemia. In Journal of genetics and genomics = Yi chuan xue bao, 46, 379-387. doi:10.1016/j.jgg.2019.06.006. https://pubmed.ncbi.nlm.nih.gov/31451425/
2. Shi, Hairong, Wang, Hongshun, Zhang, Cheng, Wei, Qinjun, Cao, Xin. 2022. Mutations in OSBPL2 cause hearing loss associated with primary cilia defects via sonic hedgehog signaling. In JCI insight, 7, . doi:10.1172/jci.insight.149626. https://pubmed.ncbi.nlm.nih.gov/35041619/
3. Li-Yang, Meina, Ma, Chao, Wang, Xiaoye, You, Jianqiang. 2024. OSBPL2 inhibition leads to apoptosis of cochlea hair cells in age-related hearing loss by inhibiting the AKT/FOXG1 signaling pathway. In Aging, 16, 13132-13144. doi:10.18632/aging.206138. https://pubmed.ncbi.nlm.nih.gov/39475791/
4. Wang, Hongshun, Lin, Changsong, Yao, Jun, Xing, Guangqian, Cao, Xin. 2019. Deletion of OSBPL2 in auditory cells increases cholesterol biosynthesis and drives reactive oxygen species production by inhibiting AMPK activity. In Cell death & disease, 10, 627. doi:10.1038/s41419-019-1858-9. https://pubmed.ncbi.nlm.nih.gov/31427568/
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