RFT1

Chr 3AR

RFT1 glycolipid translocator homolog

Also known as: CDG1N, SLC76A1

This gene encodes an enzyme that translocates dolichol-linked oligosaccharide intermediates across the endoplasmic reticulum membrane during protein N-glycosylation. Mutations cause congenital disorder of glycosylation type In, which follows autosomal recessive inheritance. The gene is not highly constrained against loss-of-function variants, consistent with the recessive inheritance pattern of this glycosylation disorder.

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 1.091 OMIM phenotype
Clinical SummaryRFT1
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Gene-Disease Validity (ClinGen)
RFT1-congenital disorder of glycosylation · ARModerate

Moderate evidence — consider for supplementary testing

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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ClinVar Variants
8 unique Pathogenic / Likely Pathogenic· 126 VUS of 300 total submissions

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.09LOEUF
pLI 0.000
Z-score 1.15
OE 0.77 (0.561.09)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
0.97Z-score
OE missense 0.84 (0.760.94)
248 obs / 294.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.77 (0.561.09)
00.351.4
Missense OE0.84 (0.760.94)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 23 / 29.8Missense obs/exp: 248 / 294.7Syn Z: 0.33
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
strongRFT1-related congenital disorder of glycosylationLOFAR

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.6745th %ile
GOF
0.6247th %ile
LOF
0.3356th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

300 submitted variants in ClinVar

Classification Summary

Pathogenic4
Likely Pathogenic4
VUS126
Likely Benign138
Benign12
Conflicting16
4
Pathogenic
4
Likely Pathogenic
126
VUS
138
Likely Benign
12
Benign
16
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
1
3
0
4
Likely Pathogenic
3
1
0
0
4
VUS
7
82
34
3
126
Likely Benign
0
7
59
72
138
Benign
0
1
11
0
12
Conflicting
16
Total109210775300

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

View in ClinVar →

Protein Context — Lollipop Plot

RFT1 · 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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