PGM3

Chr 6AR

phosphoglucomutase 3

Also known as: AGM1, IMD23, PAGM, PGM 3

The protein catalyzes the conversion of GlcNAc-6-P into GlcNAc-1-P during synthesis of UDP-GlcNAc, a sugar nucleotide critical for multiple glycosylation pathways including protein N- and O-glycosylation. Mutations cause immunodeficiency 23, an autosomal recessive disorder affecting immune system function. The gene shows low constraint against loss-of-function variants (pLI 0.000015, LOEUF 0.653).

OMIMResearchSummary from RefSeq, OMIM, UniProt
LOFmechanismARLOEUF 0.651 OMIM phenotype
Clinical SummaryPGM3
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Gene-Disease Validity (ClinGen)
immunodeficiency 23 · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

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
0.65LOEUF
pLI 0.000
Z-score 3.12
OE 0.42 (0.280.65)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.51Z-score
OE missense 0.75 (0.680.84)
227 obs / 300.7 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.42 (0.280.65)
00.351.4
Missense OE0.75 (0.680.84)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 14 / 33.5Missense obs/exp: 227 / 300.7Syn Z: 0.36
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitivePGM3-related immunodeficiencyLOFAR

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

DN
0.79top 25%
GOF
0.4776th %ile
LOF
0.2091th %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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

PGM3 · 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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