FRG1

Chr 4

FSHD region gene 1

Also known as: FRG1A, FSG1

The FRG1 protein binds mRNA and localizes to the nucleolus where it may regulate pre-mRNA splicing, ribosomal subunit assembly, and mRNA transport. Mutations cause facioscapulohumeral muscular dystrophy (FSHD), a progressive muscle disorder primarily affecting facial, shoulder, and upper arm muscles. The gene shows minimal constraint against loss-of-function variants and inheritance patterns vary depending on the specific genomic rearrangements involved in FSHD.

Summary from RefSeq, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

UniProtFacioscapulohumeral muscular dystrophy 1
0
Active trials
6
Pubs (1 yr)
0
P/LP submissions
P/LP missense
1.31
LOEUF
Mechanism
Clinical SummaryFRG1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.31LOEUF
pLI 0.000
Z-score 0.72
OE 0.79 (0.501.31)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.18Z-score
OE missense 0.95 (0.821.11)
117 obs / 122.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.79 (0.501.31)
00.351.4
Missense OE0.95 (0.821.11)
00.61.4
Synonymous OE0.88
01.21.6
LoF obs/exp: 11 / 13.9Missense obs/exp: 117 / 122.7Syn Z: 0.61

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

FRG1 · 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 →