Did you know that you can buy products from over 140 different suppliers from us?

Bardet-Biedl Syndrome 2 (BBS2) (C-Term) antibody

Details for Product No. ABIN350113
Request Want additional data for this product?

The Independent Validation Initiative strives to provide you with high quality data. Find out more

Antigen
Synonyms BBS, fb80a05, wu:fb80a05, DKFZp468B105, DKFZp469L0919
Epitope
C-Term
(2), (1), (1), (1)
Reactivity
Human
(21), (12), (12), (12), (12)
Host
Rabbit
(20), (1)
Clonality
Polyclonal
Conjugate
Un-conjugated
(1), (1), (1), (1), (1), (1), (1), (1), (1), (1), (1)
Application
Immunohistochemistry (IHC), Western Blotting (WB)
(11), (10), (4), (3), (1), (1)
Pubmed 5 references available
Quantity 100 µL
Options
Shipping to United States (Change)
Availability Will be delivered in 7 to 8 Business Days
Catalog No. ABIN350113
454.67 $
Plus shipping costs $45.00

Order hotline:

  • +1 404 474 4654
  • +1 888 205 9894 (TF)
Immunogen A synthetic peptide from c-terminal region of mouse Aquaporin 7 (AQP7) conjugated to an immunogenic carrier protein was used as the antigen.
Specificity Specific for HER2.
Purification whole serum
Alternative Name Bardet Biedl syndrome 2 protein (BBS2)
Background Function: 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 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. Defects in BBS2 are the cause of Bardet-Biedl syndrome type 2 (BBS2). Bardet-Biedl syndrome (BBS) is a genetically heterogeneous, autosomal recessive disorder 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. A relatively high incidence of BBS is found in the mixed Arab populations of Kuwait and in Bedouin tribes throughout the Middle East, most likely due to the high rate of consaguinity in these populations and a founder effect.
Subcellular location: Cell projection › cilium membrane. Cytoplasm. Note: Localizes to nonmembranous centriolar satellites in the cytoplasm.
Tissue specificity: Widely expressed. Also known as: BBS2.
UniProt P04626
Application Notes A dilution of 1 : 300 to 1 : 2000 is recommended.
The optimal dilution should be determined by the end user.
Not yet tested in other applications.
Restrictions For Research Use only
Format Liquid
Reconstitution Reconstitute in 100 µL of sterile water. Centrifuge to remove any insoluble material.
Handling Advice Avoid freeze and thaw cycles.
Storage -20 °C
Storage Comment Maintain the reconstituted antibodies frozen at -20 °C for long term storage and refrigerated at 2-8 °C for a shorter term.
Expiry Date 12 months
General Nishimura, Searby, Carmi et al.: "Positional cloning of a novel gene on chromosome 16q causing Bardet-Biedl syndrome (BBS2)." in: Human molecular genetics, Vol. 10, Issue 8, pp. 865-74, 2001 (PubMed).

Katsanis, Ansley, Badano et al.: "Triallelic inheritance in Bardet-Biedl syndrome, a Mendelian recessive disorder." in: Science (New York, N.Y.), Vol. 293, Issue 5538, pp. 2256-9, 2001 (PubMed).

Hoskins, Thorn, Scambler et al.: "Evaluation of multiplex capillary heteroduplex analysis: a rapid and sensitive mutation screening technique." in: Human mutation, Vol. 22, Issue 2, pp. 151-7, 2003 (PubMed).

Ota, Suzuki, Nishikawa et al.: "Complete sequencing and characterization of 21,243 full-length human cDNAs." in: Nature genetics, Vol. 36, Issue 1, pp. 40-5, 2003 (PubMed).

Hichri, Stoetzel, Laurier et al.: "Testing for triallelism: analysis of six BBS genes in a Bardet-Biedl syndrome family cohort." in: European journal of human genetics : EJHG, Vol. 13, Issue 5, pp. 607-16, 2005 (PubMed).

Request Want additional data for this product?

The Independent Validation Initiative strives to provide you with high quality data. Find out more

Catalog No. ABIN350113
454.67 $
Plus shipping costs $45.00

Order hotline:

  • +1 404 474 4654
  • +1 888 205 9894 (TF)
Validation Images
Did you look for something else?
back to top