BBS4 anticorps (AA 431-519) (AbBy Fluor® 350)

N° du produit ABIN1391679

Détail du produit anti-BBS4 anticorps (AbBy Fluor® 350)

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

Épitope: AA 431-519

Reactivité: Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp BBS4 est conjugé à/à la AbBy Fluor® 350

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

Réactivité croisée: Souris

Homologie: Human,Rat,Dog,Cow,Sheep,Pig,Horse

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human BBS4

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

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.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur BBS4

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

Autre désignation: BBS4 (BBS4 Produits)

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

Sujet:

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: Signalisation Hedgehog, Tube Formation, Maintenance of Protein Location