MAN2A2

Chr 15

mannosidase alpha class 2A member 2

Also known as: MANA2X, alpha-MIIx

MAN2A2 encodes an alpha-mannosidase that catalyzes the first committed step in converting high mannose to complex N-glycans in the Golgi apparatus. Mutations cause alpha-mannosidosis type II, a lysosomal storage disorder with autosomal recessive inheritance that presents with intellectual disability, hearing loss, skeletal abnormalities, and coarse facial features. The gene shows moderate constraint against loss-of-function variants.

OMIMResearchSummary from RefSeq, UniProt
LOEUF 0.62
Clinical SummaryMAN2A2
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Gene-Disease Validity (ClinGen)
congenital disorder of glycosylation · ARLimited

Limited evidence — not for standalone diagnostic reporting

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
0.62LOEUF
pLI 0.000
Z-score 3.96
OE 0.45 (0.330.62)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
1.05Z-score
OE missense 0.89 (0.830.95)
650 obs / 729.8 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.45 (0.330.62)
00.351.4
Missense OE0.89 (0.830.95)
00.61.4
Synonymous OE1.11
01.21.6
LoF obs/exp: 27 / 60.1Missense obs/exp: 650 / 729.8Syn Z: -1.45

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

MAN2A2 · 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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