ADPRHL1

Chr 13

ADP-ribosylhydrolase like 1

ADPRHL1 encodes an ADP-ribosylhydrolase that is required for myofibril assembly and cardiac chamber development, though it appears to be catalytically inactive against typical substrates. Mutations cause autosomal recessive neurodevelopmental disorder with spastic paraplegia and thin corpus callosum, affecting both the nervous system and cardiac development. This gene is highly constrained against loss-of-function variants in the population.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 1.37
Clinical SummaryADPRHL1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.37LOEUF
pLI 0.000
Z-score 0.33
OE 0.92 (0.631.37)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.34Z-score
OE missense 0.94 (0.841.05)
213 obs / 227.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.92 (0.631.37)
00.351.4
Missense OE0.94 (0.841.05)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 17 / 18.5Missense obs/exp: 213 / 227.4Syn Z: 0.56

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ADPRHL1 · 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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