ID:COG8_HUMAN DESCRIPTION: RecName: Full=Conserved oligomeric Golgi complex subunit 8; Short=COG complex subunit 8; AltName: Full=Component of oligomeric Golgi complex 8; 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. SUBCELLULAR LOCATION: Golgi apparatus membrane; Peripheral membrane protein. DISEASE: Defects in COG8 are the cause of congenital disorder of glycosylation type 2H (CDG2H) [MIM:611182]. CDGs are a family of severe inherited diseases caused by a defect in protein N- glycosylation. They are characterized by under-glycosylated serum proteins. 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. SIMILARITY: Belongs to the COG8 family. SEQUENCE CAUTION: Sequence=AAH17492.1; Type=Erroneous initiation; Sequence=BAB15301.1; Type=Frameshift; Positions=371;
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 Q96MW5
Front
Top
Side
The pictures above may be empty if there is no ModBase structure for the protein. The ModBase structure frequently covers just a fragment of the protein. You may be asked to log onto ModBase the first time you click on the pictures. It is simplest after logging in to just click on the picture again to get to the specific info on that model.
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.
AK092372 - Homo sapiens cDNA FLJ35053 fis, clone OCBBF2018362. DQ592768 - Homo sapiens piRNA piR-59880, complete sequence. DQ592767 - Homo sapiens piRNA piR-59879, complete sequence. JD135670 - Sequence 116694 from Patent EP1572962. JD135669 - Sequence 116693 from Patent EP1572962. JD181004 - Sequence 162028 from Patent EP1572962. JD051335 - Sequence 32359 from Patent EP1572962. JD217445 - Sequence 198469 from Patent EP1572962. JD408504 - Sequence 389528 from Patent EP1572962. JD195722 - Sequence 176746 from Patent EP1572962. JD194614 - Sequence 175638 from Patent EP1572962. JD194613 - Sequence 175637 from Patent EP1572962. JD143519 - Sequence 124543 from Patent EP1572962. JD417743 - Sequence 398767 from Patent EP1572962. JD417744 - Sequence 398768 from Patent EP1572962. JD126610 - Sequence 107634 from Patent EP1572962. JD293446 - Sequence 274470 from Patent EP1572962. JD246692 - Sequence 227716 from Patent EP1572962. JD084176 - Sequence 65200 from Patent EP1572962. JD182644 - Sequence 163668 from Patent EP1572962. JD331856 - Sequence 312880 from Patent EP1572962. JD175639 - Sequence 156663 from Patent EP1572962. JD138185 - Sequence 119209 from Patent EP1572962. JD417774 - Sequence 398798 from Patent EP1572962. JD143540 - Sequence 124564 from Patent EP1572962. JD548171 - Sequence 529195 from Patent EP1572962. JD421887 - Sequence 402911 from Patent EP1572962. JD533688 - Sequence 514712 from Patent EP1572962. JD446343 - Sequence 427367 from Patent EP1572962. JD023108 - Sequence 4132 from Patent EP1572962. JD030432 - Sequence 11456 from Patent EP1572962. BC019912 - Homo sapiens peptide deformylase (mitochondrial), mRNA (cDNA clone MGC:30048 IMAGE:4994286), complete cds. AF239156 - Homo sapiens peptide deformylase-like protein mRNA, complete cds. BC017492 - Homo sapiens component of oligomeric golgi complex 8, mRNA (cDNA clone IMAGE:3909241), complete cds. AK056344 - Homo sapiens cDNA FLJ31782 fis, clone NT2RI2008336, highly similar to Homo sapiens component of oligomeric golgi complex 8 (COG8), mRNA. AF322879 - Homo sapiens polypeptide deformylase-like protein (PDF) mRNA, complete cds. BC063831 - Homo sapiens cDNA clone IMAGE:6502781, **** WARNING: chimeric clone ****. AK025968 - Homo sapiens cDNA: FLJ22315 fis, clone HRC05256. AK225391 - Homo sapiens mRNA for component of oligomeric golgi complex 8 variant, clone: HRC05256. JD089909 - Sequence 70933 from Patent EP1572962. JD280891 - Sequence 261915 from Patent EP1572962. JD315217 - Sequence 296241 from Patent EP1572962. AK302605 - Homo sapiens cDNA FLJ55996 complete cds, highly similar to Conserved oligomeric Golgi complex component 8. JD361943 - Sequence 342967 from Patent EP1572962. JD360425 - Sequence 341449 from Patent EP1572962. JD274103 - Sequence 255127 from Patent EP1572962. JD470328 - Sequence 451352 from Patent EP1572962. JD382991 - Sequence 364015 from Patent EP1572962. BC121022 - Homo sapiens component of oligomeric golgi complex 8, mRNA (cDNA clone MGC:149681 IMAGE:40117369), complete cds. BC121023 - Homo sapiens component of oligomeric golgi complex 8, mRNA (cDNA clone MGC:149682 IMAGE:40117370), complete cds. KJ906364 - Synthetic construct Homo sapiens clone ccsbBroadEn_16034 COG8 gene, encodes complete protein. KJ903422 - Synthetic construct Homo sapiens clone ccsbBroadEn_12816 COG8 gene, encodes complete protein. KM576757 - Homo sapiens clone TMED6-COG8_T1-C2 mRNA sequence. KM576755 - Homo sapiens clone TMED6-COG8_T3-C2 mRNA sequence. KM576756 - Homo sapiens clone TMED6-COG8_T2-C2 mRNA sequence.
Biochemical and Signaling Pathways
Reactome (by CSHL, EBI, and GO)
Protein Q96MW5 (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
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
