AK8

Chr 9

adenylate kinase 8

Also known as: AK 8, C9orf98

Adenylate kinase 8 catalyzes the reversible transfer of phosphate groups between nucleoside triphosphates and monophosphates, with highest activity toward AMP, and is located in motile cilia where it contributes to ventricular system development. Mutations cause autosomal recessive ciliary dyskinesia with cerebral ventricular dilatation, affecting both respiratory and neurological systems. The gene is extremely intolerant to loss-of-function variants, indicating that complete loss of protein function is likely incompatible with normal development.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 1.19
Clinical SummaryAK8
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.19LOEUF
pLI 0.000
Z-score 0.85
OE 0.81 (0.561.19)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.71Z-score
OE missense 0.88 (0.790.98)
240 obs / 273.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.81 (0.561.19)
00.351.4
Missense OE0.88 (0.790.98)
00.61.4
Synonymous OE0.98
01.21.6
LoF obs/exp: 19 / 23.4Missense obs/exp: 240 / 273.2Syn Z: 0.13
DN
0.6455th %ile
GOF
0.6248th %ile
LOF
0.3161th %ile

The highest-scoring mechanism for this gene is dominant-negative.

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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

AK8 · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC