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The antibody against BBS1 was raised in rabbit using the Recombinant Human Bardet-Biedl syndrome 1 protein (217-417AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, WB, IHC, IF.
The antibody against BBS1 was raised in rabbit using the Recombinant Human Bardet-Biedl syndrome 1 protein (217-417AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, WB, IHC, IF.
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Immunohistochemistry of paraffin-embedded human kidney tissue using BBS1 Antibody at dilution of 1:1002
| Cat.No | ADC-13730A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | BBS1 |
| Form | Liquid | Species Reactivity | Human, Mouse |
| Isotype | IgG | Storage Buffer | 0.01M PBS, 0.03% Proclin 300; Constituents: 50% Glycerol, PH 7.4 |
| Purification Method | >95%, Protein G purified | Conjugate | Non-conjugated |
| Application | ELISA, IF, IHC, WB | Storage | Upon receipt |
| Immunogen Description | Recombinant Human Bardet-Biedl syndrome 1 protein (217-417AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q8NFJ9 |
Uniprot Id
Q8NFJ9
Target Species
Human
Target Name
BBS1
Target Full Name
BBSome complex member BBS1
Target 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. Plays a role in olfactory cilium biogenesis/maintenance and trafficking.
Target Involvement
Bardet-Biedl syndrome 1 (BBS1)
Target Subcellular Location
Cell projection, cilium membrane. Cytoplasm. Cytoplasm, cytoskeleton, microtubule organizing center, centrosome, centriolar satellite.
Target Tissue Specificity
Highly expressed in the kidney. Also found in fetal tissue, testis, retina, adipose tissue, heart, skeletal muscle and pancreas.
Target Synonyms
AI451249; Bardet-Biedl syndrome 1; Bardet-Biedl syndrome 1 homolog; Bardet-Biedl syndrome 1 protein; BBS1; BBS1_HUMAN; BBS2-like protein 2; BBS2L2; D19Ertd609e
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