BBS4 antibody (AA 431-519) (Alexa Fluor 350)

Catalog No. ABIN1391679

Product Details anti-BBS4 Antibody (Alexa Fluor 350)

Target: BBS4 (Bardet-Biedl Syndrome 4 (BBS4))

Binding Specificity: AA 431-519

Reactivity: Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This BBS4 antibody is conjugated to Alexa Fluor 350

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Cross-Reactivity: Mouse

Predicted Reactivity: Human,Rat,Dog,Cow,Sheep,Pig,Horse

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human BBS4

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for BBS4

Target: BBS4 (Bardet-Biedl Syndrome 4 (BBS4))

Alternative Name: BBS4 (BBS4 Products)

Synonyms: CG13232 antibody, Dmel\\CG13232 antibody, zgc:152964 antibody, AW537059 antibody, AW742241 antibody, D9Ertd464e antibody, Bardet-Biedl syndrome 4 antibody, bardet-biedl syndrome 4 antibody, Bardet-Biedl syndrome 4 (human) antibody, BBS4 antibody, bbs4 antibody, Bbs4 antibody

Background:

Synonyms: Bardet Biedl syndrome 4 protein, Bardet-Biedl syndrome 4 protein, Bbs4, BBS4_HUMAN.

Background: Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterized by obesity, photoreceptor degeneration, polydactyly, hypogenitalism, renal abnormalities, and developmental delay. Other associated clinical findings in BBS patients include diabetes, hypertension, and congenital heart defects. BBS is a heterogeneous disorder, BBS genes map to eight genetic loci and encode eight proteins, BBS1-BBS8. Five BBS genes encode basal body or cilia proteins, suggesting that BBS is a ciliary dysfunction disorder. BBS4 is expressed in the olfactory epithelium and localizes to the centriolar satellites of centrosomes and basal bodies of primary cilia. BBS4 regulates the p150 subunit of the dynein transport machinery (DCTN1) to attract pericentriolar material-1 protein (PCM1) and its associated components to the satellites. Loss of BBS4 is correlated with obesity caused by abnormal lipid profiles, liver dysfunction, elevated insulin, and abnormal leptin levels.

Pathways: Hedgehog Signaling, Tube Formation, Maintenance of Protein Location