COPB2

Chr 3q23

coat protein complex I subunit beta 2

Aliases:: beta'-COP, betaprime-COP
MANE:: ENST00000333188.10

OMIM: 606990 ↗NCBI: 9276 ↗Ensembl ↗

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Predicted protein structure

AlphaFold predicted structure

COPB2 · P35606

v62025-08-01

Predicted Aligned Error matrix for COPB2 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

86.5/ 100

Confident

906 residues

Confidence breakdown

Very high(≥ 90)61%
Confident(70–90)31%
Low(50–70)2%
Very low(< 50)7%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

DDG2P
MONOALLELIC, autosomal or pseudoautosomal, imprinted status unknown
Intellectual disability
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Osteogenesis imperfecta
MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Skeletal dysplasia
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Fetal anomalies
BOTH monoallelic and biallelic, autosomal or pseudoautosomal
Severe microcephaly
BIALLELIC, autosomal or pseudoautosomal

Disease associations (Open Targets)

osteoporosis, childhood- or juvenile-onset, with developmental delay
0.66
microcephaly 19, primary, autosomal recessive
0.52
osteoporosis
0.51
neurodegenerative disease
0.50
hypotonia with lactic acidemia and hyperammonemia
0.47
Alzheimer disease
0.46
idiopathic juvenile osteoporosis
0.46
Osteopenia
0.46
multiple sclerosis
0.46
lysosomal storage disease
0.46

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Coatomer subunit beta'

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