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Gene Review

COG1  -  component of oligomeric golgi complex 1

Homo sapiens

Synonyms: CDG2G, COG complex subunit 1, Component of oligomeric Golgi complex 1, Conserved oligomeric Golgi complex subunit 1, KIAA1381, ...


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High impact information on COG1

  • Thus, a combination of somatic cell and molecular genetics has identified a previously unrecognized protein, ldlCp, which is required for multiple Golgi functions and whose peripheral association with the Golgi is both LDLB dependent and brefeldin A sensitive [1].
  • Transfection of primary fibroblasts of this patient with the full length hemagglutinin-tagged Cog1 indeed restored beta-1,4 galactosyltransferase Golgi localization [2].
  • Here, we describe a patient with a mild form of congenital disorder of glycosylation type II (CDG-II) that is caused by a deficiency in the Cog1 subunit of the complex [2].
  • LDLB encodes an approximately 110-kDa protein, ldlBp, which lacks homology to known proteins and contains no common structural motifs [3].
  • Here, we have used a retrovirus-based expression cloning system to clone a murine cDNA, LDLB, that corrects the pleiotropic mutant phenotypes of ldlB cells [3].
  • COG1 KO HEK293T cells showed defects in Golgi morphology, retrograde trafficking and sorting, sialylation and fucosylation [4]

Associations of COG1 with chemical compounds

  • The objective of this study was to examine the interaction of apolipoprotein B (apoB) containing lipoprotein subfractions (VLDL1, Sf 60 to 400; VLDL2, Sf 20 to 60; IDL1, Sf 16 to 20; IDL2, Sf 12 to 16; LDLA, Sf 8 to 12; and LDLB, Sf 0 to 8) prepared by cumulative density gradient centrifugation with chondroitin sulfate-rich APG [5].

Analytical, diagnostic and therapeutic context of COG1

  • Sequence analysis of the COG1 cDNA and gene identified a homozygous insertion of a single nucleotide (2659-2660insC), which is predicted to lead to a premature translation stop and truncation of the C terminus of the Cog1 protein by 80 amino acids [2].
  • Immunogold electron microscopy was used to visualize the intraGolgi distribution of a functional, hemagglutinin epitope-labeled COG subunit, Cog1-HA, that complements the Cog1-deficiency in Cog1-null Chinese hamster ovary cells [6].


  1. LDLC encodes a brefeldin A-sensitive, peripheral Golgi protein required for normal Golgi function. Podos, S.D., Reddy, P., Ashkenas, J., Krieger, M. J. Cell Biol. (1994) [Pubmed]
  2. Conserved oligomeric Golgi complex subunit 1 deficiency reveals a previously uncharacterized congenital disorder of glycosylation type II. Foulquier, F., Vasile, E., Schollen, E., Callewaert, N., Raemaekers, T., Quelhas, D., Jaeken, J., Mills, P., Winchester, B., Krieger, M., Annaert, W., Matthijs, G. Proc. Natl. Acad. Sci. U.S.A. (2006) [Pubmed]
  3. Expression cloning of LDLB, a gene essential for normal Golgi function and assembly of the ldlCp complex. Chatterton, J.E., Hirsch, D., Schwartz, J.J., Bickel, P.E., Rosenberg, R.D., Lodish, H.F., Krieger, M. Proc. Natl. Acad. Sci. U.S.A. (1999) [Pubmed]
  4. COG Complex Complexities: Detailed Characterization of a Complete Set of HEK293T Cells Lacking Individual COG Subunits. Bailey Blackburn, J., Pokrovskaya, I., Fisher, P., Ungar, D., Lupashin, V.V. Front. Cell. Dev. Biol. (2016) [Pubmed]
  5. Interaction of very-low-density, intermediate-density, and low-density lipoproteins with human arterial wall proteoglycans. Anber, V., Millar, J.S., McConnell, M., Shepherd, J., Packard, C.J. Arterioscler. Thromb. Vasc. Biol. (1997) [Pubmed]
  6. IntraGolgi distribution of the Conserved Oligomeric Golgi (COG) complex. Vasile, E., Oka, T., Ericsson, M., Nakamura, N., Krieger, M. Exp. Cell Res. (2006) [Pubmed]
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