CST, 78244S, COL4A3BP (F6H3L) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying COL4A3BP. Validated for Western Blotting,Immunofluorescence (Immunocytochemistry). Available in 2 sizes. Highly specific and rigorously validated in-house, COL4A3BP (F6H3L) Rabbit Monoclonal Antibody (CST #78244) is ready to ship. Product Usage Information Western Blotting: 1:1000 Immunofluorescence (Immunocytochemistry): 1:800 Storage Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody. Protocol Available protocols: Western Blotting, Immunofluorescence (Immunocytochemistry) Specificity / Sensitivity COL4A3BP (F6H3L) Rabbit Monoclonal Antibody recognizes endogenous levels of total COL4A3BP protein. Species reactivity for immunofluorescence is human only. Species Reactivity: Human, Monkey Source / Purification Monoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues near the carboxy terminus of human COL4A3BP protein. Background Non-vesicular transport of ceramide from the endoplasmic reticulum (ER) to the Golgi requires the activity of collagen type IV alpha-3-binding protein (COL4A3BP), also known as ceramide transfer protein (CERT1) or StAR-related lipid transfer protein 11 (StARD11) (1). COL4A3BP contains an N-terminal pleckstrin homology (PH) domain that targets the protein to the Golgi, a central FFAT domain that directly binds to ER proteins VAPA and VAPB, and a C-terminal START domain that extracts ceramide from ER membranes (1-3). Phosphorylation of COL4A3BP at Ser132 by protein kinase D reduces COL4A3BP activity by inducing an autoinhibitory interaction between the PH and START domains (4,5). Sterol-sensitive activation of COL4A3BP requires desphosphorylation at Ser132 and direct interactions with both oxysterol-binding protein (OSBP) and VAPA (5,6). Silencing of COL4A3BP sensitizes cells to ER stress (7) and causes ceramide mislocalization to mitochondria, disrupting mitochondrial membranes (3). mutations, including Ser132Leu or Gly243Arg, enhance the protein's activity, causing constitutive Golgi localization and disruption of sphingolipid homeostasis (8,9). These mutations are associated with inherited intellectual disability (ID) in humans (8,9). Alternate Names C43BP; Ceramide transfer protein; ceramide transporter; ceramide transporter 1; CERT; CERT1; CERTL; COL4A3BP; collagen type IV alpha 3 binding protein; Collagen type IV alpha-3-binding protein; collagen, type IV, alpha 3 (Goodpasture antigen) binding protein; FLJ20597; Goodpasture antigen-binding protein; GPBP; hCERT; lipid-transfer protein CERTL; MRD34; StAR-related lipid transfer (START) domain containing 11; StAR-related lipid transfer protein 11; STARD11; START domain containing 11; START domain-containing protein 11 Specification REACTIVITY: H Mk SENSITIVITY: Endogenous MW (kDa): 80 Source/Isotype: Rabbit IgG