SAT1

Chr X

spermidine/spermine N1-acetyltransferase 1

Also known as: DC21, KFSD, KFSDX, SAT, SSAT, SSAT-1

The protein encoded by this gene belongs to the acetyltransferase family, and is a rate-limiting enzyme in the catabolic pathway of polyamine metabolism. It catalyzes the acetylation of spermidine and spermine, and is involved in the regulation of the intracellular concentration of polyamines and their transport out of cells. Defects in this gene are associated with keratosis follicularis spinulosa decalvans (KFSD). Alternatively spliced transcripts have been found for this gene.[provided by RefSeq, Sep 2009]

OMIMResearchGenerating clinical summary…
LOEUF 0.45
Clinical SummarySAT1
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Gene-Disease Validity (ClinGen)
pediatric systemic lupus erythematosus · XLLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.87) — some intolerance to loss-of-function variants.
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ClinVar Variants
2 VUS of 54 total submissions
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Clinical Trials
2 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.45LOEUF
pLI 0.871
Z-score 2.40
OE 0.00 (0.000.45)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
1.71Z-score
OE missense 0.40 (0.290.55)
25 obs / 63.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.00 (0.000.45)
00.351.4
Missense OE?0.40 (0.290.55)
00.61.4
Synonymous OE?1.57
01.21.6
LoF obs/exp: 0 / 6.7Missense obs/exp: 25 / 63.1Syn Z: -2.17

ClinVar Variant Classifications

54 submitted variants in ClinVar

Classification Summary

VUS2
Likely Benign2
Benign1
Conflicting1
2
VUS
2
Likely Benign
1
Benign
1
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
Likely Pathogenic
0
VUS
2
Likely Benign
2
Benign
1
Conflicting
1
Total6

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

58 pathogenic / likely-pathogenic (of 64) ClinVar copy-number / structural variants overlap SAT1 — these span large chromosomal regions, not the gene specifically, and are excluded from the counts above. Explore in CNV tools →

Protein Context — Lollipop Plot

SAT1 · protein map & ClinVar variants

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