AKAP10

Chr 17

A-kinase anchoring protein 10

Also known as: AKAP-10, D-AKAP-2, D-AKAP2, PRKA10

The AKAP10 protein binds regulatory subunits of protein kinase A and anchors them to mitochondria and plasma membranes, facilitating cellular signaling pathways including apoptosis regulation. Mutations cause autosomal dominant arrhythmogenic disorders with increased risk of cardiac arrhythmias and sudden cardiac death. This gene shows high constraint against loss-of-function variants (LOEUF 0.44), suggesting haploinsufficiency may be an important disease mechanism.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.44
Clinical SummaryAKAP10
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.25) despite low pLI — interpret in context.
📋
ClinVar Variants
98 unique Pathogenic / Likely Pathogenic· 100 VUS of 228 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.44LOEUF
pLI 0.108
Z-score 4.13
OE 0.25 (0.150.44)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.35Z-score
OE missense 0.79 (0.710.88)
263 obs / 332.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.25 (0.150.44)
00.351.4
Missense OE0.79 (0.710.88)
00.61.4
Synonymous OE1.04
01.21.6
LoF obs/exp: 9 / 35.6Missense obs/exp: 263 / 332.3Syn Z: -0.38

ClinVar Variant Classifications

228 submitted variants in ClinVar

Classification Summary

Pathogenic96
Likely Pathogenic2
VUS100
Likely Benign5
Benign3
Conflicting1
96
Pathogenic
2
Likely Pathogenic
100
VUS
5
Likely Benign
3
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
96
0
96
Likely Pathogenic
0
0
2
0
2
VUS
0
84
16
0
100
Likely Benign
0
0
1
4
5
Benign
0
2
1
0
3
Conflicting
1
Total0861164207

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

View in ClinVar →

Protein Context — Lollipop Plot

AKAP10 · 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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