FDFT1

Chr 8

farnesyl-diphosphate farnesyltransferase 1

Also known as: DGPT, ERG9, SQS, SQSD, SS

This gene encodes a membrane-associated enzyme located at a branch point in the mevalonate pathway. The encoded protein is the first specific enzyme in cholesterol biosynthesis, catalyzing the dimerization of two molecules of farnesyl diphosphate in a two-step reaction to form squalene. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtSqualene synthase deficiency

Clinical highlights

Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
2
Active trials
76
Pubs (1 yr)
P/LP submissions
P/LP missense
1.57
LOEUF
LOF
Mechanism· G2P
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GeneReview available — FDFT1
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
1.57LOEUF
pLI 0.000
Z-score -0.39
OE 1.10 (0.781.57)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-3.88Z-score
OE missense 1.71 (1.571.85)
408 obs / 239.0 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?1.10 (0.781.57)
00.351.4
Missense OE?1.71 (1.571.85)
00.61.4
Synonymous OE?2.00
01.21.6
LoF obs/exp: 21 / 19.1Missense obs/exp: 408 / 239.0Syn Z: -7.30

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FDFT1 · protein map & ClinVar variants

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