CYB5D1

Chr 17

cytochrome b5 domain containing 1

The protein is a radial spoke stalk component that binds heme and is required for coordinated ciliary beating, likely through redox signaling pathways. Mutations cause primary ciliary dyskinesia with typical respiratory symptoms and potential laterality defects. This gene follows autosomal recessive inheritance and is highly constrained against loss-of-function variants.

ResearchSummary from RefSeq, UniProt
LOEUF 1.83
Clinical SummaryCYB5D1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
22 unique Pathogenic / Likely Pathogenic· 53 VUS of 81 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.83LOEUF
pLI 0.000
Z-score -0.71
OE 1.24 (0.801.83)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.86Z-score
OE missense 0.79 (0.680.93)
107 obs / 135.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE1.24 (0.801.83)
00.351.4
Missense OE0.79 (0.680.93)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 13 / 10.5Missense obs/exp: 107 / 135.2Syn Z: 0.10

ClinVar Variant Classifications

81 submitted variants in ClinVar

Classification Summary

Pathogenic22
VUS53
Likely Benign2
22
Pathogenic
53
VUS
2
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
22
0
22
Likely Pathogenic
0
0
0
0
0
VUS
0
45
8
0
53
Likely Benign
0
2
0
0
2
Benign
0
0
0
0
0
Total04730077

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

View in ClinVar →

Protein Context — Lollipop Plot

CYB5D1 · 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 →
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