Gene: BBS10
Official Full Name: Bardet-Biedl syndrome 10provided by HGNC
Gene Summary: This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by progressive retinal degeneration, obesity, polydactyly, renal malformation and cognitive disability. The proteins encoded by BBS gene family members are structurally diverse and the similar phenotypes exhibited by mutations in BBS gene family members is likely due to their shared roles in cilia formation and function. Many BBS proteins localize to the basal bodies, ciliary axonemes, and pericentriolar regions of cells. BBS proteins may also be involved in intracellular trafficking via microtubule-related transport. The protein encoded by this gene is likely not a ciliary protein but rather has distant sequence homology to type II chaperonins. As a molecular chaperone, this protein may affect the folding or stability of other ciliary or basal body proteins. Inhibition of this protein's expression impairs ciliogenesis in preadipocytes. Mutations in this gene cause Bardet-Biedl syndrome type 10. [provided by RefSeq, Jan 2010]
Catalog Number | Product Name | Species | Gene | Passage ratio | Mycoplasma testing | Price |
---|---|---|---|---|---|---|
KO19471 | BBS10 Knockout cell line (HeLa) | Human | BBS10 | 1:3~1:6 | Negative | Online Inquiry |
KO19472 | BBS10 Knockout cell line (HCT 116) | Human | BBS10 | 1:2~1:4 | Negative | Online Inquiry |
KO19473 | BBS10 Knockout cell line (HEK293) | Human | BBS10 | 1:3~1:6 | Negative | Online Inquiry |
KO19474 | BBS10 Knockout cell line (A549) | Human | BBS10 | 1:3~1:4 | Negative | Online Inquiry |
BBS10 Gene Knockout Cell Lines are genetically modified cell lines designed to provide a robust model for studying Bardet-Biedl syndrome (BBS), a genetic disorder characterized by obesity, retinal degeneration, polydactyly, and renal anomalies. These cell lines specifically lack the BBS10 gene, which is crucial for proper ciliary function and intracellular signaling pathways. The knockout mechanism is achieved using CRISPR/Cas9 technology, a precise and efficient gene-editing tool that allows for the specific targeting and deletion of the BBS10 gene, effectively rendering its function inactive.
The key functionality of BBS10 Gene Knockout Cell Lines lies in their ability to mimic the physiological and pathological conditions of BBS, enabling researchers to investigate the molecular and cellular mechanisms underlying this complex disorder. Such studies may involve elucidating the role of cilia in cellular signaling pathways, exploring metabolic dysregulation, and evaluating potential therapeutic interventions. Moreover, these cell lines provide an invaluable resource for screening small molecules, genetic modifiers, and other therapies aimed at mitigating the effects of BBS.
The scientific significance of BBS10 Gene Knockout Cell Lines is profound, as they serve not only as experimental platforms in basic and translational research but also have potential applications in drug discovery and regenerative medicine. Compared to traditional models, such as animal studies, these cell lines offer the advantages of reduced variability, faster results, and lower ethical concerns, while also allowing for high-throughput screening and genetic manipulation.
For researchers and clinicians focused on rare genetic disorders or ciliopathies, these knockout cell lines represent a unique opportunity to deepen their understanding of BBS pathophysiology and to facilitate the development of targeted therapies. Our commitment to advancing scientific knowledge is reflected in our state-of-the-art gene-editing technologies and our extensive expertise in the field of cellular biology. BBS10 Gene Knockout Cell Lines stand as a testament to our dedication to providing innovative solutions that empower the research community.
Please note that all services are for research use only. Not intended for any clinical use.
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