SAMD12

Chr 8AD

sterile alpha motif domain containing 12

Also known as: BAFME, BAFME1, FAME, FAME1, FCMTE1, MEBA

SAMD12 encodes a protein predicted to function in cell surface receptor protein tyrosine kinase signaling at the cytoplasmic side of the plasma membrane. Mutations cause familial adult myoclonic epilepsy type 1, which follows autosomal dominant inheritance with adult onset as the name suggests. The gene shows tolerance to loss-of-function variants (pLI 0.001, LOEUF 1.14), indicating that complete loss of protein function may not be the primary disease mechanism.

Summary from RefSeq, OMIM
Research Assistant →

Primary Disease Associations & Inheritance

Epilepsy, familial adult myoclonic, 1MIM #601068
AD
0
Active trials
9
Pubs (1 yr)
61
P/LP submissions
0%
P/LP missense
1.14
LOEUF
Multiple*
Mechanism· predicted
Clinical SummarySAMD12
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
61 unique Pathogenic / Likely Pathogenic· 37 VUS of 119 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.14LOEUF
pLI 0.001
Z-score 1.26
OE 0.58 (0.321.14)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.08Z-score
OE missense 1.02 (0.881.20)
109 obs / 106.6 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.58 (0.321.14)
00.351.4
Missense OE1.02 (0.881.20)
00.61.4
Synonymous OE1.08
01.21.6
LoF obs/exp: 6 / 10.4Missense obs/exp: 109 / 106.6Syn Z: -0.39
DN
0.6744th %ile
GOF
0.6540th %ile
LOF
0.3842th %ile

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

DNprediction above median
GOFprediction 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

119 submitted variants in ClinVar

Classification Summary

Pathogenic61
VUS37
Likely Benign5
61
Pathogenic
37
VUS
5
Likely Benign

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
61
0
61
Likely Pathogenic
0
0
0
0
0
VUS
0
28
9
0
37
Likely Benign
0
3
2
0
5
Benign
0
0
0
0
0
Total031720103

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SAMD12 · 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.

Search ClinicalTrials.gov →
Clinical Literature
Open Research Assistant →