ATP11B

Chr 3

ATPase phospholipid transporting 11B (putative)

Also known as: ATPIF, ATPIR

P-type ATPases, such as ATP11B, are phosphorylated in their intermediate state and drive uphill transport of ions across membranes. Several subfamilies of P-type ATPases have been identified. One subfamily transports heavy metal ions, such as Cu(2+) or Cd(2+). Another subfamily transports non-heavy metal ions, such as H(+), Na(+), K(+), or Ca(+). A third subfamily transports amphipaths, such as phosphatidylserine.[supplied by OMIM, Feb 2005]

OMIMResearchGenerating clinical summary…
MultiplemechanismLOEUF 0.41
Clinical SummaryATP11B
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?
0.41LOEUF
pLI 0.002
Z-score 5.56
OE 0.28 (0.190.41)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
1.85Z-score
OE missense 0.79 (0.730.85)
477 obs / 604.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.28 (0.190.41)
00.351.4
Missense OE?0.79 (0.730.85)
00.61.4
Synonymous OE?0.91
01.21.6
LoF obs/exp: 19 / 68.7Missense obs/exp: 477 / 604.7Syn Z: 0.98

This gene — mechanism propensity

DN
0.6840th %ile
GOF
0.74top 25%
LOF
0.2679th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

ATP11B · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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