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C57BL/6JCya-Bbs4em1/Cya
Common Name:
Bbs4-KO
Product ID:
S-KO-18212
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Bbs4-KO
Strain ID
KOCMP-102774-Bbs4-B6J-VA
Gene Name
Bbs4
Product ID
S-KO-18212
Gene Alias
D9Ertd464e
Background
C57BL/6JCya
NCBI ID
102774
Modification
Conventional knockout
Chromosome
9
Phenotype
MGI:2143311
Document
Click here to download >>
Application
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Rare Disease Data Center >>
Note
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Bbs4em1/Cya mice (Catalog S-KO-18212) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000026265
NCBI RefSeq
NM_175325
Target Region
Exon 2
Size of Effective Region
~0.8 kb
Detailed Document
Click here to download >>
Overview of Gene Research
Bbs4 is a gene encoding a protein involved in Bardet-Biedl syndrome (BBS), an autosomal recessive ciliopathy. BBS proteins localize to cilia and basal bodies, participating in cilia biogenesis and function [1].

In Bbs4 -/- mice, olfactory sensory neuron (OSN) cilia are shorter and fewer, with asynchronous rates of intraflagellar transport (IFT)-A/B particle movements, indicating miscoordination in IFT complex trafficking. Also, BBS4 loss leads to a reduction in basal body numbers in OSNs [2].

In adipocytes, Bbs4 silencing in 3T3F442A pre-adipocytes induces accelerated cell division and aberrant differentiation, resulting in more triglyceride accumulation [4]. BBS4 silencing in neuronal cells reduces sensitivity to endoplasmic reticulum (ER) stress during differentiation and under ER-stress induction, as it is essential for the nuclear transport of transcription factors sXBP-1 and ATF6α p50 [3].

In conclusion, Bbs4 plays crucial roles in cilia-related functions in olfactory neurons, and in the regulation of adipocyte proliferation, differentiation, and ER stress response in adipocytes and neuronal cells. The study of Bbs4 knockout mouse models has provided insights into its functions in the context of ciliopathies, obesity, and neural development-related diseases [2,3,4].

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. 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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