ERICH5

Chr 8

glutamate rich 5

Also known as: C8orf47

ERICH5 encodes a protein containing glutamate-rich and histidine-rich domains whose specific cellular function remains unclear. Mutations cause autosomal recessive intellectual disability with seizures and dysmorphic features, typically manifesting in early childhood. The gene shows high tolerance to loss-of-function variants in the general population, consistent with a recessive inheritance pattern.

LOEUF 1.21
Clinical SummaryERICH5
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
34 unique Pathogenic / Likely Pathogenic· 50 VUS of 88 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.21LOEUF
pLI 0.000
Z-score 1.03
OE 0.69 (0.421.21)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.15Z-score
OE missense 1.03 (0.921.16)
199 obs / 192.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.69 (0.421.21)
00.351.4
Missense OE1.03 (0.921.16)
00.61.4
Synonymous OE0.90
01.21.6
LoF obs/exp: 9 / 13.0Missense obs/exp: 199 / 192.9Syn Z: 0.64

ClinVar Variant Classifications

88 submitted variants in ClinVar

Classification Summary

Pathogenic33
Likely Pathogenic1
VUS50
Likely Benign4
33
Pathogenic
1
Likely Pathogenic
50
VUS
4
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
33
0
33
Likely Pathogenic
0
0
1
0
1
VUS
0
48
2
0
50
Likely Benign
0
4
0
0
4
Benign
0
0
0
0
0
Total05236088

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

View in ClinVar →

Protein Context — Lollipop Plot

ERICH5 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →
Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC

No open access results found