- Antibody type:Primary
- Antigen name:BBS4
- Clonality:Polyclonal
- Conjugation:Alexa Fluor® 680
- Host:Rabbit
- ImmunoChemistry:Yes
- ImmunoFluorescence:Yes
- Isotype:IgG
- Reactivity:Mouse
- Western blot:Yes
- Epitope:431-519/519
- Form:Liquid
- Antigen synonyms:Bbs4|Bardet Biedl syndrome 4 protein|BBS4_HUMAN|Bardet-Biedl syndrome 4 protein
- Modification:Unmodified
- Storage buffer:Aqueous buffered solution containing 0,01 M TBS (pH 7,4) with 1% BSA, 0,03% Proclin300 and 50% Glycerol
- Molecular weight:58 kDa
- Storage temperature:Store at −20 °C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
- Concentration:1 µg/µl
- Shipping temperature:4 °C
- Immunogen:KLH conjugated synthetic peptide derived from human BBS4
- Tested applications:ICC
- Purification:Purified by Protein A
- Pack type:Vial
- Pk:100 µl
Bardet-Biedl syndrome (BBS) is a pleiotropic genetic disorder characterised 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.
Type: Primary
Antigen: BBS4
Clonality: Polyclonal
Clone:
Conjugation: ALEXA FLUOR® 680
Public Immunogen Range: 431-519/519
Host: Rabbit
Isotype: IgG
Reactivity: Mouse