Aliases for COPZ1 Gene
External Ids for COPZ1 Gene
Previous HGNC Symbols for COPZ1 Gene
Previous GeneCards Identifiers for COPZ1 Gene
This gene encodes a subunit of the cytoplasmic coatamer protein complex, which is involved in autophagy and intracellular protein trafficking. The coatomer protein complex is comprised of seven subunits and functions as the coat protein of coat protein complex (COP)I-vesicles. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Nov 2012]
GeneCards Summary for COPZ1 Gene
COPZ1 (Coatomer Protein Complex Subunit Zeta 1) is a Protein Coding gene. Diseases associated with COPZ1 include Neurodegeneration With Brain Iron Accumulation 6. Among its related pathways are wtCFTR and delta508-CFTR traffic / Generic schema (norm and CF) and Golgi-to-ER retrograde transport. An important paralog of this gene is COPZ2.
UniProtKB/Swiss-Prot for COPZ1 Gene
The coatomer is a cytosolic protein complex that binds to dilysine motifs and reversibly associates with Golgi non-clathrin-coated vesicles, which further mediate biosynthetic protein transport from the ER, via the Golgi up to the trans Golgi network. Coatomer complex is required for budding from Golgi membranes, and is essential for the retrograde Golgi-to-ER transport of dilysine-tagged proteins. In mammals, the coatomer can only be recruited by membranes associated to ADP-ribosylation factors (ARFs), which are small GTP-binding proteins; the complex also influences the Golgi structural integrity, as well as the processing, activity, and endocytic recycling of LDL receptors (By similarity).
The zeta subunit may be involved in regulating the coat assembly and, hence, the rate of biosynthetic protein transport due to its association-dissociation properties with the coatomer complex.