A2ML1

Chr 12AD

alpha-2-macroglobulin like 1

Also known as: CPAMD9, OMS, p170

This gene encodes a member of the alpha-macroglobulin superfamily. The encoded protein is thought to be an N-glycosylated monomeric protein that acts as an inhibitor of several proteases. It has been shown to form covalent interactions with proteases, and has been reported as the p170 antigen recognized by autoantibodies in the autoimmune disease paraneoplastic pemphigus (PNP; PMID:20805888). Mutations in these gene have also been associated with some cases of Noonan syndrome (NS; PMID:24939586) as well as some cases of otitis media (PMID:26121085). Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Aug 2015]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

{Otitis media, susceptibility to}MIM #166760
AD

Clinical highlights

Gene-disease validity (ClinGen)
Noonan syndrome · ADDisputedevidence questions this relationship
0
Active trials
11
Pubs (1 yr)
P/LP submissions
P/LP missense
0.95
LOEUF
Mechanism
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GeneReview available — A2ML1
Authoritative clinical overview · Recommended first read
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Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.95LOEUF
pLI 0.000
Z-score 1.91
OE 0.77 (0.630.95)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.33Z-score
OE missense 0.97 (0.911.03)
766 obs / 791.9 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.77 (0.630.95)
00.351.4
Missense OE?0.97 (0.911.03)
00.61.4
Synonymous OE?1.00
01.21.6
LoF obs/exp: 63 / 81.6Missense obs/exp: 766 / 791.9Syn Z: -0.07

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

A2ML1 · 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.

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