bbs-9 Antibody

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Description

BBS-9 Antibody Overview

The BBS-9 antibody is a polyclonal rabbit IgG that recognizes specific epitopes of the BBS9 protein, which plays a structural and functional role in the BBSome—an octameric complex essential for trafficking membrane proteins to primary cilia . Key features include:

ParameterDetails
TargetBBS9 (UniProt: Q3SYG4 in humans, Q811G0 in mice)
HostRabbit
ClonalityPolyclonal
ImmunogenSynthetic peptides or recombinant fragments (e.g., N-terminal, residues 1–200)
ReactivityHuman, mouse, rat, cow, dog, horse, pig, rabbit, guinea pig
ApplicationsWestern blot (WB), immunohistochemistry (IHC), immunofluorescence (IF/ICC), immunoprecipitation (IP), ELISA

Key Applications

  • Western Blot: Detects BBS9 at ~60–99 kDa across tissues (e.g., human heart, mouse testis) .

  • Immunofluorescence: Localizes BBS9 to primary cilia in hTERT-RPE1 cells .

  • Immunohistochemistry: Strong cytoplasmic staining in Purkinje cells (human cerebellum) .

  • Functional Studies: Used to investigate BBS9’s role in osteogenic differentiation and ciliogenesis .

Recommended Dilutions

ApplicationDilution Range
WB1:500–1:1000
IHC1:20–1:200
IF/ICC1:10–1:100
IP0.5–4.0 µg per 1–3 mg lysate

Disease Associations

  • Bardet-Biedl Syndrome (BBS): Pathogenic mutations (e.g., L518P, N524Δ in BBS1) perturb BBS9 interactions, impairing ciliary trafficking .

  • Craniosynostosis: Upregulated BBS9 splice variants disrupt osteogenic differentiation in calvarial sutures .

  • Ciliopathies: Knockdown of BBS9 in zebrafish causes hydrocephalus and shortened cilia, mimicking human BBS phenotypes .

Functional Studies

  • Cargo Transport: BBS9 facilitates Rab8-GTP localization to cilia, enabling vesicle docking .

  • Protein Stability: The G182A mutation reduces BBS9 stability, accelerating degradation .

Critical Considerations

  • Sodium Azide Warning: Some formulations contain sodium azide, requiring careful handling .

  • Validation: Optimal dilutions vary by cell/tissue type; empirical testing is recommended .

  • Species Cross-Reactivity: Predicted reactivity with cow (100%), dog (100%), and pig (100%) based on sequence homology .

Product Specs

Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Form
Liquid
Lead Time
Made-to-order (14-16 weeks)
Synonyms
bbs-9 antibody; C48B6.8 antibody; Protein pthb1 homolog antibody; Bardet-Biedl syndrome 9 protein homolog antibody
Target Names
bbs-9
Uniprot No.

Target Background

Function
BBS-9 Antibody is a component of the BBSome complex. The BBSome complex is believed to act as a coat complex essential for the sorting of specific membrane proteins to the primary cilia. This complex is crucial for ciliogenesis but is not required for centriolar satellite function. BBS-9 Antibody is required for proper BBSome complex assembly and its ciliary localization. It plays a key role in cilia biogenesis and both the assembly and movement of intraflagellar transport proteins along the ciliary axoneme. In ciliated sensory neurons, BBS-9 Antibody is necessary for the sensation of nitric oxide and avoidance of NO-producing organisms such as P.aeruginosa.
Database Links

KEGG: cel:CELE_C48B6.8

STRING: 6239.C48B6.8

UniGene: Cel.26121

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