Anti-BBS4 antibody (bs-11508R)

Anti-BBS4 antibody

DATASHEET

Host:Rabbit

Target Protein:BBS4

IR:Immunogen Range:431-519/519

Clonality:Polyclonal

Isotype:IgG

Entrez Gene:585

Swiss Prot:Q96RK4

Source:KLH conjugated synthetic peptide derived from human BBS4:431-519/519 

Purification:affinity purified by Protein A

Storage:0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

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.

Size:100ul

Concentration:1mg/ml

Applications:WB(1:500-2000)
ELISA(1:5000-10000)
IHC-P(1:100-500)
IHC-F(1:100-500)
ICC(1:100-500)
IF(1:100-500)

Cross Reactive Species:Human
Mouse
Rat
Dog
Pig
Cow
Horse
Sheep
.

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Sample: TM4 Cell (Mouse) Lysate at 40 ug
Primary: Anti-BBS4 (bs-11508R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 58 kD
Observed band size: 58 kD

Sample: Adrenal gland (Mouse) Lysate at 40 ug
Primary: Anti-BBS4 (bs-11508R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 58 kD
Observed band size: 58 kD