COG4

Multiprotein complexes are key determinants of Golgi apparatus structure and its capacity for intracellular transport and glycoprotein modification. Several complexes have been identified, including the Golgi transport complex (GTC), the LDLC complex, which is involved in glycosylation reactions, and the SEC34 complex, which is involved in vesicular transport. These 3 complexes are identical and have been termed the conserved oligomeric Golgi (COG) complex, which includes COG4 (Ungar et al., 2002 [PubMed 11980916]).[supplied by OMIM

Full Name

component of oligomeric golgi complex 4

Function

Required for normal Golgi function (PubMed:19536132, PubMed:30290151).

Plays a role in SNARE-pin assembly and Golgi-to-ER retrograde transport via its interaction with SCFD1 (PubMed:19536132).

Biological Process

Endoplasmic reticulum to Golgi vesicle-mediated transport Source: Reactome
Golgi organization Source: UniProtKB
Golgi vesicle prefusion complex stabilization Source: UniProtKB
Protein transport Source: UniProtKB-KW
Retrograde vesicle-mediated transport, Golgi to endoplasmic reticulum Source: UniProtKB

Cellular Location

Cytosol; Golgi apparatus membrane. Mosty cytosolic, with about 5% membrane-bound.

Involvement in disease

Congenital disorder of glycosylation 2J (CDG2J):
A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in 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.
Saul-Wilson syndrome (SWILS):
A rare skeletal dysplasia with characteristic dysmorphic and radiographic findings, as well as early developmental delay, primarily involving speech, with eventual normal cognition. Clinical findings include marked short stature, prominent forehead with an enlarged anterior fontanel, prominent eyes with cataracts, narrow nasal bridge with a convex nasal ridge, micrognathia, clubfoot, brachydactyly, and short distal phalanges of fingers. Radiographic changes include platyspondyly, irregular end plates of vertebral bodies, and hypoplasia of the odontoid process with cervical instability in the spine, coxa valga, overtubulation, metaphyseal flaring and megaepiphyses in the long bones, while the hands and feet exhibit short phalanges, metacarpals and metatarsals, cone-shaped epiphyses of phalanges, and accessory ossification centers of metacarpals and metatarsals.