BBS4 Antikörper (AA 431-519) (AbBy Fluor® 647)

Produktnummer ABIN1391682

Dieses Anti-BBS4-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von BBS4 in WB, IF (cc) und IF (p). Geeignet für Maus.

Kurzübersicht für BBS4 Antikörper (AA 431-519) (AbBy Fluor® 647) (ABIN1391682)

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

Reaktivität: Maus

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser BBS4 Antikörper ist konjugiert mit AbBy Fluor® 647

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

Produktdetails anti-BBS4 Antikörper (AbBy Fluor® 647)

Bindungsspezifität: AA 431-519

Kreuzreaktivität: Maus

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

Aufreinigung: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human BBS4

Isotyp: IgG

Anwendungsinformationen

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

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 1 μg/μL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Haltbarkeit: 12 months

Details zu BBS4

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

Andere Bezeichnung: BBS4

Hintergrund:

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 Signalweg, Tube Formation, Maintenance of Protein Location