The Coat Protein Complex II, or COPII, is a group of proteins that facilitate the formation of vesicles to transport proteins from the endoplasmic reticulum to the Golgi apparatus or endoplasmic-reticulum–Golgi intermediate compartment. This process is termed anterograde transport, in contrast to the retrograde transport associated with the COPI complex. COPII is assembled in two parts: first an inner layer of Sar1, Sec23, and Sec24 forms; then the inner coat is surrounded by an outer lattice of Sec13 and Sec31.
Homocysteine-responsive endoplasmic reticulum-resident ubiquitin-like domain member 1 protein is a protein that in humans is encoded by the HERPUD1 gene.
Protein transport protein Sec61 subunit beta is a protein that in humans is encoded by the SEC61B gene.
Ubiquitin-conjugating enzyme E2 G2 is a protein that in humans is encoded by the UBE2G2 gene.
Protein SEC13 homolog is a protein that in humans is encoded by the SEC13 gene.
KDEL (Lys-Asp-Glu-Leu) endoplasmic reticulum protein retention receptor 1, also known as KDELR1, is a protein which in humans is encoded by the KDELR1 gene.
Alpha-1,3/1,6-mannosyltransferase ALG2 is an enzyme that is encoded by the ALG2 gene. Mutations in the human gene are associated with congenital defects in glycosylation The protein encoded by the ALG2 gene belongs to two classes of enzymes: GDP-Man:Man1GlcNAc2-PP-dolichol alpha-1,3-mannosyltransferase and GDP-Man:Man2GlcNAc2-PP-dolichol alpha-1,6-mannosyltransferase.
Ribosome-binding protein 1, also referred to as p180, is a protein that in humans is encoded by the RRBP1 gene.
Conserved oligomeric Golgi complex subunit 3 is a protein that in humans is encoded by the COG3 gene.
Protein CLN8 is a protein that in humans is encoded by the CLN8 gene.
Nucleotide exchange factor SIL1 is a protein that in humans is encoded by the SIL1 gene.
Translocon-associated protein subunit beta also known as TRAP-beta is a protein that in humans is encoded by the SSR2 gene.
ER lumen protein retaining receptor 2 is a protein that in humans is encoded by the KDELR2 gene.
Vesicle-trafficking protein SEC22a is a protein that in humans is encoded by the SEC22A gene.
Protein transport protein Sec24C is a protein that in humans is encoded by the SEC24C gene.
Protein transport protein Sec24D is a protein that in humans is encoded by the SEC24D gene.
Protein transport protein Sec24B is a protein that in humans is encoded by the SEC24B gene.
SEC23-interacting protein is a protein that in humans is encoded by the SEC23IP gene.
SEC31 is a protein which in yeast promotes the formation of COPII transport vesicles from the Endoplasmic Reticulum (ER). The coat has two main functions, the physical deformation of the endoplasmic reticulum membrane into vesicles and the selection of cargo molecules.
Halperin-Birk syndrome (HLBKS) is a rare autosomal recessive neurodevelopmental disorder caused by a null mutation in the SEC31A gene. Signs and symptoms include intrauterine growth retardation, marked developmental delay, spastic quadriplegia with profound contractures, dysmorphism, and optic nerve atrophy with no eye fixation. Brain MRI demonstrated microcephaly and agenesis of the corpus callosum.
