ARPP21

Chr 3

cAMP regulated phosphoprotein 21

Also known as: ARPP-21, R3HDM3, RCS, TARPP

The protein is a cAMP-regulated phosphoprotein enriched in the caudate nucleus and cerebellar cortex that may inhibit calmodulin-dependent enzymes such as calcineurin in neurons and regulate dopamine effects in the basal ganglia. Mutations cause autosomal recessive neurodevelopmental disorders affecting the brain. The gene is highly constrained against loss-of-function variants, indicating that complete loss of protein function is likely not tolerated.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.44
Clinical SummaryARPP21
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Gene-Disease Validity (ClinGen)
amyotrophic lateral sclerosis · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.28) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.44LOEUF
pLI 0.012
Z-score 4.60
OE 0.28 (0.180.44)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.26Z-score
OE missense 0.97 (0.891.04)
432 obs / 447.5 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.28 (0.180.44)
00.351.4
Missense OE0.97 (0.891.04)
00.61.4
Synonymous OE0.92
01.21.6
LoF obs/exp: 13 / 47.1Missense obs/exp: 432 / 447.5Syn Z: 0.83
DN
0.7033th %ile
GOF
0.5367th %ile
LOF
0.49top 25%

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

ARPP21 · 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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