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ACMG/AMP Criteria Reference

This page summarizes the clinical meaning and current execution status of each ACMG/AMP criterion. Exact application depends on variant type, disease mechanism, inheritance, source quality, applicable ClinGen guidance, and the evidence available for the case.

Status labels

Automated means the production pipeline can evaluate the criterion from governed data. Clinical review means the evidence depends on case-level judgment or information not inferred by the pipeline. Governed means an implementation exists but activation depends on validated reference data and deployment configuration.

Pathogenic Evidence

PVS1Very Strong, with supported modificationsAutomated

Predicted loss of function where loss of function is an established disease mechanism

PS1StrongAutomated

Same amino acid change as an established pathogenic variant

PS2StrongClinical review

De novo occurrence with confirmed parental relationships

PS3StrongClinical review

Well-established functional evidence supports a damaging effect

PS4StrongClinical review

Increased prevalence in affected individuals

PM1ModerateAutomated

Location in a critical functional region without benign variation

PM2Moderate or SupportingAutomated

Absent or sufficiently rare in population reference data

PM3ModerateAutomated

Observed in trans with a pathogenic partner in a recessive disorder

PM4ModerateAutomated

Protein length change in an applicable non-repeat context

PM5ModerateGoverned

Different pathogenic missense change at the same residue

PM6ModerateClinical review

Assumed de novo occurrence without confirmed parental relationships

PP1Supporting, with supported modificationsClinical review

Cosegregation with disease in affected family members

PP2SupportingAutomated

Missense change in a gene where missense variation is an established disease mechanism

PP3Supporting, Moderate, or StrongAutomated

Calibrated computational evidence supports a damaging effect

PP4SupportingAutomated

Patient phenotype is highly specific for the associated disorder or gene

PP5SupportingAutomated

A reputable clinical source reports pathogenicity

Benign Evidence

BA1Stand-aloneAutomated

Population frequency is incompatible with the disorder

BS1Strong, with supported modificationsAutomated

Population frequency is greater than expected for the disorder

BS2StrongAutomated

Observed in healthy individuals where full penetrance is expected

BS3StrongClinical review

Well-established functional evidence supports no damaging effect

BS4StrongClinical review

Lack of segregation with disease

BP1SupportingAutomated

Missense change in a gene where truncating variation is the established mechanism

BP2SupportingAutomated

Allelic observation is inconsistent with the proposed disease mechanism

BP3SupportingAutomated

In-frame length change in a repetitive region without known function

BP4Supporting or ModerateAutomated

Calibrated computational evidence supports no damaging effect

BP5SupportingClinical review

An alternate molecular basis explains the case

BP6SupportingAutomated

A reputable clinical source reports benignity

BP7SupportingAutomated

Synonymous change with no predicted splice impact

Framework boundary

This reference describes nuclear sequence-variant criteria. Mitochondrial and structural variants use dedicated specifications that include, exclude, or modify criteria for their biology and evidence model.