COPZ2

Chr 17

coat protein complex I subunit zeta 2

Also known as: zeta2-COP

The protein functions as the zeta subunit of the coatomer complex, which is essential for retrograde Golgi-to-ER transport of dilysine-tagged proteins and biosynthetic protein transport through the secretory pathway. The gene has extremely low constraint scores (pLI and LOEUF), indicating tolerance to loss-of-function variants, and no specific diseases associated with COPZ2 mutations are currently established in the provided data.

Summary from RefSeq, UniProt
0
Active trials
3
Pubs (1 yr)
9
P/LP submissions
0%
P/LP missense
1.61
LOEUF
Mechanism
Clinical SummaryCOPZ2
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
9 unique Pathogenic / Likely Pathogenic· 2 VUS of 19 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.61LOEUF
pLI 0.000
Z-score 0.03
OE 0.99 (0.621.61)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.12Z-score
OE missense 1.03 (0.881.22)
102 obs / 98.7 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.99 (0.621.61)
00.351.4
Missense OE1.03 (0.881.22)
00.61.4
Synonymous OE0.61
01.21.6
LoF obs/exp: 11 / 11.1Missense obs/exp: 102 / 98.7Syn Z: 1.90

ClinVar Variant Classifications

19 submitted variants in ClinVar

Classification Summary

Pathogenic8
Likely Pathogenic1
VUS2
Likely Benign1
8
Pathogenic
1
Likely Pathogenic
2
VUS
1
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
8
0
8
Likely Pathogenic
0
0
1
0
1
VUS
0
1
1
0
2
Likely Benign
0
1
0
0
1
Benign
0
0
0
0
0
Total0210012

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

COPZ2 · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Key Publications
Landmark & review papers · by relevance
PubMed
Top 1 results · since 2015Search PubMed ↗
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC