Mouse Anti-BBS2 Recombinant Antibody (CBYY-0253) (CBMAB-0254-YY)

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Published Data

Mouse Anti-BBS2 Recombinant Antibody (CBYY-0253) (CBMAB-0254-YY) in IHC

Retinal histology of 4-month-old Bbs2−/−, Bbs7−/−, Bbs8−/−, Bbs4−/− and Bbs3−/− mice and their control littermates was visualized by light microscopy, and the thicknesses of their outer nuclear layers were quantified. ...View More

Hsu, Y., Seo, S., & Sheffield, V. C. (2021). Photoreceptor cilia, in contrast to primary cilia, grant entry to a partially assembled BBSome. Human molecular genetics, 30(1), 87-102.

Mouse Anti-BBS2 Recombinant Antibody (CBYY-0253) (CBMAB-0254-YY) in IP

Coimmunoprecipitation (IP) of the BBSome subunits from YFP-BBS4 WT RPE1
cell lines using anti-GFP antibodies before and after 24-h serum starvation. ...View More

Prasai, A., Cernohorska, M. S., Ruppova, K., Niederlova, V., Andelova, M., Draber, P., ... & Huranova, M. (2020). The BBSome assembly is spatially controlled by BBS1 and BBS4 in human cells. Journal of Biological Chemistry, 295(42), 14279-14290.

Mouse Anti-BBS2 Recombinant Antibody (CBYY-0253) (CBMAB-0254-YY) in IF

Wild-type, Ift27, Lztfl1, and Bbs2 mutant MEFs were transfected with PD23 (SmoM2-Flag) or GP735 (SmoM2-Flag-Ub) and selected with Bsd. Confluent cells were serum starved for 48 h before being fixed and stained for SmoM2 or SmoM2-Ub (Flag; red), cilia (Arl13b; green; arrows), and nuclei (DAPI; blue). Left image of each pair is a three-color composite; right image shows only the red Flag channel. Scale bar is 5 μm and applies to all images in the panel. ...View More

Desai, P. B., Stuck, M. W., Lv, B., & Pazour, G. J. (2020). Ubiquitin links smoothened to intraflagellar transport to regulate Hedgehog signaling. Journal of Cell Biology, 219(7), e201912104.

Mouse Anti-BBS2 Recombinant Antibody (CBYY-0253) (CBMAB-0254-YY) in IF

ARPE-19 cells transfected with control (siNSp) or Cep290 (siCep290) siRNA and plasmid expressing an irrelevant Flag-tagged protein (control) or full-length Flag-NPHP5 (1-598), induced to quiescence, were stained with antibodies against Flag (green), detyrosinated-tubulin or GT335 (blue), and BBS2, BBS5 or BBS8 (red). ...View More

Barbelanne, M., Hossain, D., Chan, D. P., Peränen, J., & Tsang, W. Y. (2015). Nephrocystin proteins NPHP5 and Cep290 regulate BBSome integrity, ciliary trafficking and cargo delivery. Human molecular genetics, 24(8), 2185-2200.

Datasheet

Summary

Host Animal

Mouse

Specificity

Human, Mouse, Rat

Clone

CBYY-0253

Antibody Isotype

IgG1, κ

Application

WB, IP, IF, ELISA

Basic Information

Immunogen

Amino acids 55-300 mapping near the N-terminus of BBS2 of human origin.

Host Species

Mouse

Specificity

Human, Mouse, Rat

Antibody Isotype

IgG1, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
WB	1:100-1:1,000
IP	1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
IF(ICC)	1:50-1:500
ELISA	1:100-1:1,000

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

PBS, 0.1% gelatin

Preservative

< 0.1% sodium azide

Concentration

0.2 mg/ml

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Epitope

N-Terminus

Target

Full Name

Bardet-Biedl Syndrome 2

Introduction

This gene is a member of the Bardet-Biedl syndrome (BBS) gene family. Bardet-Biedl syndrome is an autosomal recessive disorder characterized by severe pigmentary retinopathy, 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 forMouse a multiprotein BBSome complex with seven other BBS proteins.[provided by RefSeq, Oct 2014]

Entrez Gene ID

Human	583
Dog	478121

UniProt ID

Human	Q9BXC9
Dog	E2RRL8

Alternative Names

Bardet-Biedl Syndrome 2; Bardet-Biedl Syndrome 2 Protein; RP74; BBS;

Function

The BBSome complex is thought to function as a coat complex required for sorting of specific membrane proteins to the primary cilia. The BBSome complex is required for ciliogenesis but is dispensable for centriolar satellite function. This ciliogenic function is mediated in part by the Rab8 GDP/GTP exchange factor, which localizes to the basal body and contacts the BBSome. Rab8(GTP) enters the primary cilium and promotes extension of the ciliary membrane. Firstly the BBSome associates with the ciliary membrane and binds to RAB3IP/Rabin8, the guanosyl exchange factor (GEF) for Rab8 and then the Rab8-GTP localizes to the cilium and promotes docking and fusion of carrier vesicles to the base of the ciliary membrane. The BBSome complex, together with the LTZL1, controls SMO ciliary trafficking and contributes to the sonic hedgehog (SHH) pathway regulation. Required for proper BBSome complex assembly and its ciliary localization.

Biological Process

Adult behavior Source: BHF-UCL
Artery smooth muscle contraction Source: Ensembl
Brain morphogenesis Source: BHF-UCL
Cartilage development Source: Ensembl
Cerebral cortex development Source: BHF-UCL
Cilium assembly Source: BHF-UCL
Fat cell differentiation Source: BHF-UCL
Golgi to plasma membrane protein transport Source: MGI
Hippocampus development Source: BHF-UCL
Melanosome transport Source: BHF-UCL
Negative regulation of appetite by leptin-mediated signaling pathway Source: BHF-UCL
Negative regulation of gene expression Source: Ensembl
Negative regulation of multicellular organism growth Source: BHF-UCL
Non-motile cilium assembly Source: Ensembl
Photoreceptor cell maintenance Source: BHF-UCL
Positive regulation of multicellular organism growth Source: Ensembl
Protein localization Source: BHF-UCL
Protein localization to organelle Source: BHF-UCL
Regulation of cilium beat frequency involved in ciliary motility Source: BHF-UCL
Sperm axoneme assembly Source: BHF-UCL
Striatum development Source: BHF-UCL
Vasodilation Source: Ensembl
Visual perception Source: UniProtKB

Cellular Location

Cytoplasm; Cilium membrane; Centriolar satellite

Involvement in disease

Bardet-Biedl syndrome 2 (BBS2): A syndrome characterized by usually severe pigmentary retinopathy, early-onset obesity, polydactyly, hypogenitalism, renal malformation and mental retardation. Secondary features include diabetes mellitus, hypertension and congenital heart disease. Bardet-Biedl syndrome inheritance is autosomal recessive, but three mutated alleles (two at one locus, and a third at a second locus) may be required for clinical manifestation of some forms of the disease.
Retinitis pigmentosa 74 (RP74): A retinal dystrophy belonging to the group of pigmentary retinopathies. Retinitis pigmentosa is characterized by retinal pigment deposits visible on fundus examination and primary loss of rod photoreceptor cells followed by secondary loss of cone photoreceptors. Patients typically have night vision blindness and loss of midperipheral visual field. As their condition progresses, they lose their far peripheral visual field and eventually central vision as well.

More Infomation

References

Ali, G., Foo, J. N., Nasir, A., Chang, C. H., Chew, E. G., Latif, Z., ... & Nisar, R. (2021). Identification of a Novel Homozygous Missense (c. 443A> T: p. N148I) Mutation in BBS2 in a Kashmiri Family with Bardet-Biedl Syndrome. BioMed Research International, 2021.

Huang, L., Sun, L., Wang, Z., Li, S., Chen, C., Luo, X., & Ding, X. (2021). Novel Compound Heterozygous BBS2 and Homozygous MKKS Variants Detected in Chinese Families with Bardet–Biedl Syndrome. Journal of Ophthalmology, 2021.

Ting, C. Y., Huang, C. Y., Chen, H. C., Chiu, Y. W., Hsieh, P. C., & Lee, J. J. (2021). Generation of induced pluripotent stem cells from a Bardet-Biedl syndrome patient carrying a homologous BBS2 c. 534+ 1G> T mutation. Stem Cell Research, 55, 102480.

Perkins, B., Song, P., Fogerty, J., Cianciolo, L., & Stupay, R. (2020). Cone photoreceptor degeneration and neuroinflammation in the zebrafish Bardet-Biedl syndrome 2 (bbs2) mutant does not lead to retinal regeneration. Frontiers in cell and developmental biology, 8, 1410.

Ludlam, W. G., Aoba, T., Cuéllar, J., Bueno-Carrasco, M. T., Makaju, A., Moody, J. D., ... & Willardson, B. M. (2019). Molecular architecture of the Bardet–Biedl syndrome protein 2-7-9 subcomplex. Journal of Biological Chemistry, 294(44), 16385-16399.

Zahid, S., Branham, K., Schlegel, D., Pennesi, M. E., Michaelides, M., Heckenlively, J., & Jayasundera, T. (2018). BBS2. In Retinal Dystrophy Gene Atlas (pp. 19-21). Springer, Cham.

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Protocols

Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme-Linked Immunospot (ELISpot)
Proteogenomics
Other Protocols

Associated Products

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Isotype control

For research use only. Not intended for any clinical use.

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