ID:COG1_HUMAN DESCRIPTION: RecName: Full=Conserved oligomeric Golgi complex subunit 1; Short=COG complex subunit 1; AltName: Full=Component of oligomeric Golgi complex 1; FUNCTION: Required for normal Golgi function (By similarity). SUBUNIT: Component of the conserved oligomeric Golgi complex which is composed of eight different subunits and is required for normal Golgi morphology and localization. INTERACTION: Q9H9E3:COG4; NbExp=2; IntAct=EBI-368371, EBI-368382; SUBCELLULAR LOCATION: Golgi apparatus membrane; Peripheral membrane protein; Cytoplasmic side (Probable). DISEASE: Defects in COG1 are the cause of congenital disorder of glycosylation type 2G (CDG2G) [MIM:611209]; also known as CDG-II caused by COG1 deficiency. CDGs are a family of severe inherited diseases caused by a defect in glycoprotein biosynthesis. They are characterized by under-glycosylated serum glycoproteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Clinical features of CDG2G include failure to thrive, generalized hypotonia, growth retardation and mild psychomotor retardation. CDG2G is biochemically characterized by a defect in O-glycosylation as well as N-glycosylation. SIMILARITY: Belongs to the COG1 family. SEQUENCE CAUTION: Sequence=BAA92619.1; Type=Frameshift; Positions=937;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
ModBase Predicted Comparative 3D Structure on Q8WTW3
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
Biological Process: GO:0006888 ER to Golgi vesicle-mediated transport GO:0006891 intra-Golgi vesicle-mediated transport GO:0007030 Golgi organization GO:0015031 protein transport
BC047465 - Homo sapiens component of oligomeric golgi complex 1, mRNA (cDNA clone MGC:51244 IMAGE:5285077), complete cds. AK098224 - Homo sapiens cDNA FLJ40905 fis, clone UTERU2004664, highly similar to Conserved oligomeric Golgi complex component 1. DQ896029 - Synthetic construct Homo sapiens clone IMAGE:100010489; FLH189669.01L; RZPDo839H0964D component of oligomeric golgi complex 1 (COG1) gene, encodes complete protein. AB462989 - Synthetic construct DNA, clone: pF1KA1381, Homo sapiens COG1 gene for component of oligomeric golgi complex 1, without stop codon, in Flexi system. BC021985 - Homo sapiens component of oligomeric golgi complex 1, mRNA (cDNA clone MGC:2368 IMAGE:2821581), complete cds. AB037802 - Homo sapiens mRNA for KIAA1381 protein, partial cds. AK025633 - Homo sapiens cDNA: FLJ21980 fis, clone HEP06118. AL359611 - Homo sapiens mRNA; cDNA DKFZp762L1710 (from clone DKFZp762L1710). LF209047 - JP 2014500723-A/16550: Polycomb-Associated Non-Coding RNAs. JD410801 - Sequence 391825 from Patent EP1572962. MA444624 - JP 2018138019-A/16550: Polycomb-Associated Non-Coding RNAs.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein Q8WTW3 (Reactome details) participates in the following event(s):
R-HSA-6809006 Vesicle is tethered through binding GOLGA2:GORASP1, GOLGB1 and the COG complex R-HSA-6811433 The COG tethering complex interacts with numerous SNAREs at the Golgi membrane R-HSA-8847544 The COG complex and CUX1 and GOLGA5 dimers contribute to intra-Golgi vesicle tethering R-HSA-8849748 The COG complex binds RABs at the Golgi membrane R-HSA-6811431 RAB6:GTP binds the GARP and COG complexes, t-SNAREs and endosome-derived vesicles R-HSA-6809011 cis-Golgi t-SNAREs bind YKT6 on tethered vesicle R-HSA-6807878 COPI-mediated anterograde transport R-HSA-6811438 Intra-Golgi traffic R-HSA-6811440 Retrograde transport at the Trans-Golgi-Network R-HSA-199977 ER to Golgi Anterograde Transport R-HSA-6811442 Intra-Golgi and retrograde Golgi-to-ER traffic R-HSA-199991 Membrane Trafficking R-HSA-948021 Transport to the Golgi and subsequent modification R-HSA-5653656 Vesicle-mediated transport R-HSA-446203 Asparagine N-linked glycosylation R-HSA-597592 Post-translational protein modification R-HSA-392499 Metabolism of proteins
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Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
