ACP5

Chr 19AR

acid phosphatase 5, tartrate resistant

Also known as: HPAP, TRACP5a, TRACP5b, TRAP, TRAcP, TrATPase

The protein is an iron-containing acid phosphatase that catalyzes dephosphorylation of bone matrix proteins including osteopontin and bone sialoprotein. Mutations cause spondyloenchondrodysplasia with immune dysregulation, a skeletal dysplasia with immunodeficiency that follows autosomal recessive inheritance. The gene shows moderate constraint against loss-of-function variants with a LOEUF score of 0.724.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.721 OMIM phenotype
Clinical SummaryACP5
Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.32) despite low pLI — interpret in context.
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.72LOEUF
pLI 0.079
Z-score 2.25
OE 0.32 (0.150.72)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
0.40Z-score
OE missense 0.92 (0.811.04)
181 obs / 196.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.32 (0.150.72)
00.351.4
Missense OE0.92 (0.811.04)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 4 / 12.6Missense obs/exp: 181 / 196.8Syn Z: 0.56
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveACP5-related spondyloenchondrodysplasia with immune dysregulationLOFAR

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

DN
0.6937th %ile
GOF
0.6248th %ile
LOF
0.2775th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ACP5 · 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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