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Gene Review

COL4A3BP  -  collagen, type IV, alpha 3 (Goodpasture...

Homo sapiens

Synonyms: CERT, CERTL, Ceramide transfer protein, Collagen type IV alpha-3-binding protein, GPBP, ...
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Disease relevance of COL4A3BP


High impact information on COL4A3BP


Biological context of COL4A3BP

  • In human tissues, GPBP shows a more limited expression than GPBPDelta26 but displays a remarkable preference for the small vessels and for histological structures targeted by natural autoimmune responses including alveolar and glomerular basement membranes, the two main targets in Goodpasture disease [1].
  • We have recently cloned from a HeLa-derived cDNA library a novel serine/threonine kinase (Goodpasture antigen-binding protein (GPBP)) that phosphorylates the N-terminal region of the human domain (Raya, A. Revert, F, Navarro, S. and Saus J. (1999) J. Biol. Chem. 274, 12642-12649) [1].
  • CERT expressed in LY-A cells has an amino acid substitution that destroys its PtdIns4P-binding activity, thereby impairing its Golgi-targeting function [6].

Anatomical context of COL4A3BP

  • The recently identified ceramide transfer protein, CERT, is responsible for the bulk of ceramide transport from the endoplasmic reticulum (ER) to the Golgi [7].
  • Children with ECC at their initial examination (N = 58) had a 93.0% cert rate, a 67.2% CERT rate and a 60.3% CERT in first molars rate by their last dental examination [8].

Associations of COL4A3BP with chemical compounds

  • We show here that the pre-mRNA of GPBP is alternatively spliced in human tissues and that the most common transcript found encodes GPBPDelta26, a molecular isoform devoid of a 26-residue serine-rich motif [1].
  • The main pathway of endoplasmic reticulum-to-Golgi transport of ceramide is mediated by CERT, a cytosolic 68-kDa protein, in a nonvesicular manner [9].
  • CERT did not mediate the transfer of sphingosine or sphingomyelin at all [9].
  • CERT, which is identical to a splicing variant of Goodpasture antigen-binding protein, is a cytoplasmic protein with a phosphatidylinositol-4-monophosphate-binding (PtdIns4P) domain and a putative domain for catalysing lipid transfer [6].
  • CERT has a C-terminal START domain for ceramide binding and an N-terminal pleck-strin homology domain that binds phosphatidylinositol 4-phosphate suggesting that phosphatidylinositol (PI) 4-kinases are involved in the regulation of CERT-mediated ceramide transport [7].

Other interactions of COL4A3BP


Analytical, diagnostic and therapeutic context of COL4A3BP


  1. Goodpasture antigen-binding protein, the kinase that phosphorylates the goodpasture antigen, is an alternatively spliced variant implicated in autoimmune pathogenesis. Raya, A., Revert-Ros, F., Martinez-Martinez, P., Navarro, S., Rosello, E., Vieites, B., Granero, F., Forteza, J., Saus, J. J. Biol. Chem. (2000) [Pubmed]
  2. The effects of the morphological response of Enterobacteriaceae to cephalosporins on PAE and CERT. Mackenzie, F.M., Milne, K.E., Gould, I.M. Scand. J. Infect. Dis. (1998) [Pubmed]
  3. Inter-organelle membrane contact sites: through a glass, darkly. Levine, T., Loewen, C. Curr. Opin. Cell Biol. (2006) [Pubmed]
  4. Oxysterol-binding protein and vesicle-associated membrane protein-associated protein are required for sterol-dependent activation of the ceramide transport protein. Perry, R.J., Ridgway, N.D. Mol. Biol. Cell (2006) [Pubmed]
  5. Efficient Trafficking of Ceramide from the Endoplasmic Reticulum to the Golgi Apparatus Requires a VAMP-associated Protein-interacting FFAT Motif of CERT. Kawano, M., Kumagai, K., Nishijima, M., Hanada, K. J. Biol. Chem. (2006) [Pubmed]
  6. Molecular machinery for non-vesicular trafficking of ceramide. Hanada, K., Kumagai, K., Yasuda, S., Miura, Y., Kawano, M., Fukasawa, M., Nishijima, M. Nature (2003) [Pubmed]
  7. Phosphatidylinositol 4-Kinase IIIbeta Regulates the Transport of Ceramide between the Endoplasmic Reticulum and Golgi. T??th, B., Balla, A., Ma, H., Knight, Z.A., Shokat, K.M., Balla, T. J. Biol. Chem. (2006) [Pubmed]
  8. Primary incisor decay before age 4 as a risk factor for future dental caries. al-Shalan, T.A., Erickson, P.R., Hardie, N.A. Pediatric dentistry. (1997) [Pubmed]
  9. CERT mediates intermembrane transfer of various molecular species of ceramides. Kumagai, K., Yasuda, S., Okemoto, K., Nishijima, M., Kobayashi, S., Hanada, K. J. Biol. Chem. (2005) [Pubmed]
  10. A human-specific TNF-responsive promoter for Goodpasture antigen-binding protein. Granero, F., Revert, F., Revert-Ros, F., Lainez, S., Martínez-Martínez, P., Saus, J. FEBS J. (2005) [Pubmed]
  11. Characterization of a novel type of serine/threonine kinase that specifically phosphorylates the human goodpasture antigen. Raya, A., Revert, F., Navarro, S., Saus, J. J. Biol. Chem. (1999) [Pubmed]
  12. Cetuximab combined with radiotherapy: An alternative to chemoradiotherapy for patients with locally advanced squamous cell carcinomas of the head and neck? Bernier, J., Schneider, D. Eur. J. Cancer (2007) [Pubmed]
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