ADK

Chr 10AR

adenosine kinase

Also known as: AK

Adenosine kinase phosphorylates adenosine to adenosine monophosphate in an ATP-dependent manner and plays a key role in detoxifying modified adenosines containing methylated adenine that arise from RNA degradation. Mutations cause hypermethioninemia due to adenosine kinase deficiency, which follows autosomal recessive inheritance. The gene shows minimal constraint against loss-of-function variants (pLI 0.0003, LOEUF 0.798).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.801 OMIM phenotype
Clinical SummaryADK
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Gene-Disease Validity (ClinGen)
adenosine kinase deficiency · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
18 unique Pathogenic / Likely Pathogenic· 35 VUS of 105 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.80LOEUF
pLI 0.000
Z-score 2.23
OE 0.46 (0.280.80)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.50Z-score
OE missense 0.69 (0.600.80)
129 obs / 186.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.46 (0.280.80)
00.351.4
Missense OE0.69 (0.600.80)
00.61.4
Synonymous OE0.87
01.21.6
LoF obs/exp: 9 / 19.7Missense obs/exp: 129 / 186.9Syn Z: 0.80
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongADK-related intellectual developmental disorderLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7035th %ile
GOF
0.5661th %ile
LOF
0.2872th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

105 submitted variants in ClinVar

Classification Summary

Pathogenic15
Likely Pathogenic3
VUS35
Likely Benign33
Benign16
Conflicting3
15
Pathogenic
3
Likely Pathogenic
35
VUS
33
Likely Benign
16
Benign
3
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
1
3
11
0
15
Likely Pathogenic
2
1
0
0
3
VUS
1
17
16
1
35
Likely Benign
0
2
22
9
33
Benign
0
1
14
1
16
Conflicting
3
Total4246311105

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

View in ClinVar →

Protein Context — Lollipop Plot

ADK · 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.

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Clinical Literature
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