C57BL/6JCya-Bbs4em1flox/Cya
Common Name
Bbs4-flox
Product ID
S-CKO-00299
Backgroud
C57BL/6JCya
Strain ID
CKOCMP-102774-Bbs4-B6J-VA
Status
When using this mouse strain in a publication, please cite “Bbs4-flox Mouse (Catalog S-CKO-00299) 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
Bbs4-flox
Strain ID
CKOCMP-102774-Bbs4-B6J-VA
Gene Name
Product ID
S-CKO-00299
Gene Alias
D9Ertd464e
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
Chr 9 (Mouse)
Phenotype
Datasheet
Application
--
Strain Description
Ensembl Transcript ID
ENSMUST00000026265
NCBI Transcript ID
NM_175325
Target Region
Exon 2
Size of Effective Region
~0.8 kb
Overview of Gene Research
Bbs4 is one of the genes associated with Bardet-Biedl syndrome (BBS), a rare autosomal recessive ciliopathy. The BBS genes encode proteins localizing to cilia and basal bodies, involved in cilia biogenesis and function [1]. Bbs4 is crucial for normal cilia-related functions and may also have extra-ciliary roles, and its study using genetic models can help understand its functions in vivo.
In Bbs4 -/- mice, olfactory sensory neuron (OSN) cilia are shorter and fewer, with asynchronous intraflagellar transport (IFT) particle movements, indicating miscoordination in IFT complex trafficking. Also, basal body numbers are reduced. Adenoviral expression of BBS4 can restore OSN cilia lengths and odor detection but not ciliary and basal body numbers [2]. In adipocytes, silencing Bbs4 leads to accelerated cell division, aberrant differentiation, and greater triglyceride accumulation. Bbs4 is also involved in adipocyte endoplasmic reticulum (ER) stress response, with its depletion resulting in swollen ER and impaired nuclear translocation of XBP-1 and ATF6α [4,5]. In neuronal cells, BBS4 silencing reduces sensitivity to ER stress during differentiation, as sXBP-1 and activated ATF6α p50 fail to translocate to the nucleus [3].
In conclusion, Bbs4 is essential for cilia-related functions in olfactory neurons and plays significant roles in adipocyte and neuronal development and stress responses. Studies using Bbs4 knockout mouse models have provided insights into the mechanisms underlying BBS-associated phenotypes such as obesity, olfactory dysfunction, and potential neural development abnormalities.
References：
1. Forsythe, Elizabeth, Beales, Philip L. 2012. Bardet-Biedl syndrome. In European journal of human genetics : EJHG, 21, 8-13. doi:10.1038/ejhg.2012.115. https://pubmed.ncbi.nlm.nih.gov/22713813/
2. Uytingco, Cedric R, Williams, Corey L, Xie, Chao, Sheffield, Val C, Martens, Jeffrey R. 2019. BBS4 is required for intraflagellar transport coordination and basal body number in mammalian olfactory cilia. In Journal of cell science, 132, . doi:10.1242/jcs.222331. https://pubmed.ncbi.nlm.nih.gov/30665891/
3. Horwitz, Avital, Birk, Ruth. 2020. BBS4 Is Essential for Nuclear Transport of Transcription Factors Mediating Neuronal ER Stress Response. In Molecular neurobiology, 58, 78-91. doi:10.1007/s12035-020-02104-z. https://pubmed.ncbi.nlm.nih.gov/32894499/
4. Anosov, Mariana, Birk, Ruth. 2019. Bardet-Biedl syndrome obesity: BBS4 regulates cellular ER stress in early adipogenesis. In Molecular genetics and metabolism, 126, 495-503. doi:10.1016/j.ymgme.2019.03.006. https://pubmed.ncbi.nlm.nih.gov/30902542/
5. Aksanov, Olga, Green, Pnina, Birk, Ruth Z. 2014. BBS4 directly affects proliferation and differentiation of adipocytes. In Cellular and molecular life sciences : CMLS, 71, 3381-92. doi:10.1007/s00018-014-1571-x. https://pubmed.ncbi.nlm.nih.gov/24500759/
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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