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Page 1
BBS4 Is Essential for Nuclear Transport of Transcription Factors Mediating Neuronal ER Stress Response.
Horwitz A, Birk R. Horwitz A, et al. Mol Neurobiol. 2021 Jan;58(1):78-91. doi: 10.1007/s12035-020-02104-z. Epub 2020 Sep 7. Mol Neurobiol. 2021. PMID: 32894499
Hence, BBS4 is essential for nuclear transport under ER-stress response in neuronal cells during early differentiation. Our studies shed light on molecular mechanisms through which BBS4 malfunction alters neuronal ER stress response....
Hence, BBS4 is essential for nuclear transport under ER-stress response in neuronal cells during early differentiation. Our studies s …
BBS4 protein has basal body/ciliary localization in sensory organs but extra-ciliary localization in oligodendrocytes during human development.
Bénardais K, Delfino G, Samama B, Devys D, Antal MC, Ghandour MS, Boehm N. Bénardais K, et al. Cell Tissue Res. 2021 Jul;385(1):37-48. doi: 10.1007/s00441-021-03440-9. Epub 2021 Apr 16. Cell Tissue Res. 2021. PMID: 33860840
We observed a correlation between the differentiation of the neurosensory cells (hair cells, photoreceptors, olfactory neurons) and the presence of a punctate BBS4 immunostaining in their apical cytoplasm. In the brain, BBS4 was localized in oligodendrocytes and mye …
We observed a correlation between the differentiation of the neurosensory cells (hair cells, photoreceptors, olfactory neurons) and the pres …
Bardet-Biedl syndrome.
Forsythe E, Beales PL. Forsythe E, et al. Eur J Hum Genet. 2013 Jan;21(1):8-13. doi: 10.1038/ejhg.2012.115. Epub 2012 Jun 20. Eur J Hum Genet. 2013. PMID: 22713813 Free PMC article.
Bardet-Biedl Syndrome Overview.
Forsyth R, Gunay-Aygun M. Forsyth R, et al. 2003 Jul 14 [updated 2023 Mar 23]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024. 2003 Jul 14 [updated 2023 Mar 23]. In: Adam MP, Feldman J, Mirzaa GM, Pagon RA, Wallace SE, Bean LJH, Gripp KW, Amemiya A, editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993–2024. PMID: 20301537 Free Books & Documents. Review.
BBS4 is required for intraflagellar transport coordination and basal body number in mammalian olfactory cilia.
Uytingco CR, Williams CL, Xie C, Shively DT, Green WW, Ukhanov K, Zhang L, Nishimura DY, Sheffield VC, Martens JR. Uytingco CR, et al. J Cell Sci. 2019 Feb 15;132(5):jcs222331. doi: 10.1242/jcs.222331. J Cell Sci. 2019. PMID: 30665891 Free PMC article.
Here, we report that Bbs4(-/-) mice exhibit shorter and fewer olfactory sensory neuron (OSN) cilia despite retaining odorant receptor localization. Within Bbs4(-/-) OSN cilia, we observed asynchronous rates of IFT-A/B particle movements, indicating miscoordination i …
Here, we report that Bbs4(-/-) mice exhibit shorter and fewer olfactory sensory neuron (OSN) cilia despite retaining odorant receptor …
BBS4 directly affects proliferation and differentiation of adipocytes.
Aksanov O, Green P, Birk RZ. Aksanov O, et al. Cell Mol Life Sci. 2014 Sep;71(17):3381-92. doi: 10.1007/s00018-014-1571-x. Epub 2014 Feb 6. Cell Mol Life Sci. 2014. PMID: 24500759 Free PMC article.
BBS4 polymorphisms have been associated with common non-syndromic morbid obesity. BBS4 obesity molecular mechanisms, and the role of the BBS4 gene in adipocyte differentiation and function are not entirely known. ...
BBS4 polymorphisms have been associated with common non-syndromic morbid obesity. BBS4 obesity molecular mechanisms, and the r …
Bardet-Biedl syndrome obesity: BBS4 regulates cellular ER stress in early adipogenesis.
Anosov M, Birk R. Anosov M, et al. Mol Genet Metab. 2019 Apr;126(4):495-503. doi: 10.1016/j.ymgme.2019.03.006. Epub 2019 Mar 15. Mol Genet Metab. 2019. PMID: 30902542
We verified BBS4's ER localization in adipocytes by immunocytochemistry and cellular protein fractionation. ...BBS4 depletion results in swollen ER with impaired intracellular nucleus translocation of XBP-1 and ATF6alpha. ...
We verified BBS4's ER localization in adipocytes by immunocytochemistry and cellular protein fractionation. ...BBS4 depletion …
Insulin regulates Bbs4 during adipogenesis.
Nahum N, Forti E, Aksanov O, Birk R. Nahum N, et al. IUBMB Life. 2017 Jul;69(7):489-499. doi: 10.1002/iub.1626. Epub 2017 Apr 3. IUBMB Life. 2017. PMID: 28371235 Free article.
Silencing of Bbs4 expression in adipocytes significantly impaired and reduced glucose uptake. This effect was reversed by Bbs4 overexpression. Inhibition of PI 3-kinase resulted in upregulation of Bbs transcripts, suggesting that the PI3K pathway is involved in the …
Silencing of Bbs4 expression in adipocytes significantly impaired and reduced glucose uptake. This effect was reversed by Bbs4 …
A BBS4 mutation causes autosomal dominant polycystic liver disease.
Cui Y, Xu W, Liu J, Liu S, Huang W, Shi Y, Zhang X, Lu C, Xie W. Cui Y, et al. Genes Dis. 2023 Apr 5;11(1):72-75. doi: 10.1016/j.gendis.2023.02.042. eCollection 2024 Jan. Genes Dis. 2023. PMID: 37588201 Free PMC article. No abstract available.
BBS4 regulates the expression and secretion of FSTL1, a protein that participates in ciliogenesis and the differentiation of 3T3-L1.
Prieto-Echagüe V, Lodh S, Colman L, Bobba N, Santos L, Katsanis N, Escande C, Zaghloul NA, Badano JL. Prieto-Echagüe V, et al. Sci Rep. 2017 Aug 29;7(1):9765. doi: 10.1038/s41598-017-10330-0. Sci Rep. 2017. PMID: 28852127 Free PMC article.
Importantly, understanding both cilia and cilia-independent functions of the BBS proteins is key to fully dissect the cellular basis of the syndrome. Here we characterize a functional interaction between BBS4 and the secreted protein FSTL1, a protein linked to adipogenesis …
Importantly, understanding both cilia and cilia-independent functions of the BBS proteins is key to fully dissect the cellular basis of the …
153 results