LAMA2

Chr 6AR

laminin subunit alpha 2

Also known as: LAMM, MDC1A

The LAMA2 gene encodes the alpha-2 chain of laminin, an extracellular matrix protein that forms a major component of basement membranes and mediates cell attachment and organization during development. Mutations cause congenital merosin-deficient muscular dystrophy and limb-girdle muscular dystrophy type 23, both inherited in an autosomal recessive pattern. The pathogenic mechanism involves loss of function of the laminin protein, disrupting the structural integrity of muscle basement membranes.

OMIMResearchSummary from RefSeq, OMIM, UniProt, Mechanism
LOFmechanismARLOEUF 0.732 OMIM phenotypes
Clinical SummaryLAMA2
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Gene-Disease Validity (ClinGen)
LAMA2-related muscular dystrophy · 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.
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Clinical Trials
4 active or recruiting trials — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.73LOEUF
pLI 0.000
Z-score 4.50
OE 0.62 (0.530.73)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.03Z-score
OE missense 1.00 (0.961.04)
1698 obs / 1694.5 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.62 (0.530.73)
00.351.4
Missense OE1.00 (0.961.04)
00.61.4
Synonymous OE1.07
01.21.6
LoF obs/exp: 102 / 164.3Missense obs/exp: 1698 / 1694.5Syn Z: -1.44
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
definitiveLAMA2-related congenital muscular dystrophyLOFAR

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

DN
0.7228th %ile
GOF
0.6930th %ile
LOF
0.2385th %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

LAMA2 · protein map & ClinVar variants

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

3D Protein StructureAlphaFold
Clinical Literature
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