FREM1

Chr 9ARAD

FRAS1 related extracellular matrix 1

FREM1 encodes an extracellular matrix protein required for epidermal adhesion during embryonic development and plays a role in epidermal differentiation. Mutations cause bifid nose with or without anorectal and renal anomalies, Manitoba oculotrichoanal syndrome, and trigonocephaly 2, with both autosomal recessive and autosomal dominant inheritance patterns reported. These conditions primarily affect craniofacial development, with variable involvement of renal and anorectal systems.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismAR/ADLOEUF 1.123 OMIM phenotypes
Clinical SummaryFREM1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
Some data sources returned errors (1)

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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.12LOEUF
pLI 0.000
Z-score 0.61
OE 0.93 (0.781.12)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-3.51Z-score
OE missense 1.29 (1.241.35)
1485 obs / 1149.9 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.93 (0.781.12)
00.351.4
Missense OE1.29 (1.241.35)
00.61.4
Synonymous OE1.30
01.21.6
LoF obs/exp: 83 / 89.2Missense obs/exp: 1485 / 1149.9Syn Z: -5.00

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FREM1 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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