BBS1 anticorps (AA 11-275)

N° du produit ABIN7870073

Cet anticorps Lapin Polyclonal détecte spécifiquement BBS1 dans ELISA et WB. Il présente une réactivité avec des échantillons de Humain, Souris et Rat.

Aperçu rapide pour BBS1 anticorps (AA 11-275) (ABIN7870073)

Antigène: BBS1 (Bardet-Biedl Syndrome 1 (BBS1))

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp BBS1 est non-conjugé

Application: ELISA, Western Blotting (WB)

Détail du produit anti-BBS1 anticorps

Épitope: AA 11-275

Fonction: BBS1 Antibody / Bardet-Biedl syndrome 1

Purification: Immunogen affinity purified

Immunogène: E.coli-derived human ATF5 recombinant protein (Position: L11-R275) was used as the immunogen for the BBS1 antibody.

Isotype: IgG

Information d'application

Indications d'application: Optimal dilution of the BBS1 antibody should be determined by the researcher.

Restrictions: For Research Use only

Stockage

Format: Lyophilized

Reconstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Stock: 4 °C,-20 °C

Stockage commentaire: After reconstitution, the BBS1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Détails sur BBS1

Antigène: BBS1 (Bardet-Biedl Syndrome 1 (BBS1))

Autre désignation: BBS1

Sujet: The BBS1 antibody targets Bardet-Biedl syndrome 1 protein, a centrosomal and ciliary component encoded by the BBS1 gene. This protein is a core subunit of the BBSome, a multimeric complex required for the trafficking of membrane proteins to and from primary cilia. Bardet-Biedl syndrome 1 protein plays a crucial role in ciliary signaling, cell polarity, and sensory perception. The BBS1 antibody provides a key reagent for studying cilia function, intracellular transport, and ciliopathies associated with BBSome dysfunction.

The BBSome complex, composed of at least eight core proteins including BBS1, BBS2, BBS4, and BBS7, mediates cargo recognition and vesicular transport along the ciliary membrane. Bardet-Biedl syndrome 1 protein acts as a structural hub that anchors the complex to the basal body and interacts with small GTPases such as ARL6. The BBS1 antibody allows visualization of this component in centrosomes and cilia, supporting studies into how BBSome assembly regulates signaling receptor localization and function.

Mutations in BBS1 are the most common cause of Bardet-Biedl syndrome, a pleiotropic ciliopathy characterized by retinal degeneration, obesity, renal anomalies, and polydactyly. These mutations impair BBSome stability and disrupt ciliary transport of G-protein-coupled receptors (GPCRs). The BBS1 antibody supports functional research into these mechanisms by enabling analysis of protein expression, localization, and complex formation in patient cells and model organisms. It is a critical tool for defining how ciliary trafficking defects lead to multisystemic disease manifestations.

Beyond ciliopathy research, Bardet-Biedl syndrome 1 protein has been implicated in metabolic regulation, immune signaling, and neural development. The BBS1 antibody supports studies exploring these expanded roles, including how BBSome components modulate leptin receptor and Hedgehog signaling pathways. Dysregulation of ciliary protein transport impacts sensory and hormonal signaling, linking BBS1 to diverse physiological processes.

The BBS1 antibody performs effectively in western blotting, immunofluorescence, and immunohistochemistry, revealing punctate centrosomal and ciliary staining patterns. NSJ Bioreagents provides this antibody with validated specificity for reproducible detection across mammalian models. By enabling precise analysis of Bardet-Biedl syndrome 1 protein, the BBS1 antibody supports ongoing research into ciliary trafficking, BBSome structure, and the molecular basis of human ciliopathies.

UniProt: Q8NFJ9

Pathways: Signalisation Hedgehog