CCR4

Chr 3

C-C motif chemokine receptor 4

Also known as: CC-CKR-4, CD194, CKR4, CMKBR4, ChemR13, HGCN:14099, K5-5

The encoded protein is a phosphatase that converts NADP+ to NAD+ and regulates post-transcriptional control of metabolic genes under circadian control, playing key roles in nutrient metabolism, adipogenesis, and early embryonic development. Mutations cause autosomal recessive neurodevelopmental disorders with intellectual disability, seizures, and metabolic dysfunction. The gene shows low constraint to loss-of-function variants, consistent with recessive inheritance patterns.

GeneReviewsOMIMResearchSummary from RefSeq, UniProt
MultiplemechanismLOEUF 0.97
Clinical SummaryCCR4
Population Constraint (gnomAD)
Low constraint (pLI 0.07) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
16 unique Pathogenic / Likely Pathogenic· 36 VUS of 54 total submissions
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Clinical Trials
4 active or recruiting trials — potential therapeutic options may be available
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GeneReview available — CCR4
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.97LOEUF
pLI 0.069
Z-score 1.63
OE 0.38 (0.170.97)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.75Z-score
OE missense 0.85 (0.750.97)
164 obs / 193.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.38 (0.170.97)
00.351.4
Missense OE0.85 (0.750.97)
00.61.4
Synonymous OE1.25
01.21.6
LoF obs/exp: 3 / 8.0Missense obs/exp: 164 / 193.2Syn Z: -1.74
DN
0.80top 25%
GOF
0.80top 10%
LOF
0.1993th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
DNprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

54 submitted variants in ClinVar

Classification Summary

Pathogenic16
VUS36
Likely Benign2
16
Pathogenic
36
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
16
0
16
Likely Pathogenic
0
0
0
0
0
VUS
0
33
3
0
36
Likely Benign
0
2
0
0
2
Benign
0
0
0
0
0
Total03519054

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

View in ClinVar →

Protein Context — Lollipop Plot

CCR4 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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