EFEMP2

Chr 11

EGF-like fibulin extracellular matrix protein 2

Also known as: ARCL1B, FBLN4, MBP1, UPH1

A large number of extracellular matrix proteins have been found to contain variations of the epidermal growth factor (EGF) domain and have been implicated in functions as diverse as blood coagulation, activation of complement and determination of cell fate during development. The protein encoded by this gene contains four EGF2 domains and six calcium-binding EGF2 domains. This gene is necessary for elastic fiber formation and connective tissue development. Defects in this gene are cause of an autosomal recessive cutis laxa syndrome. Alternatively spliced transcript variants have been identified for this gene. [provided by RefSeq, Jan 2011]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtCutis laxa, autosomal recessive, 1B

Clinical highlights

Gene-disease validity (ClinGen)
cutis laxa, autosomal recessive, type 1B · ARDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype) and the gene is intolerant of it in the population — truncating, frameshift and canonical splice variants carry more prior weight here than missense.Curated gene-level mechanism — a prior for triage, not a per-variant call.
0
Active trials
16
Pubs (1 yr)
P/LP submissions
P/LP missense
0.60
LOEUF
LOF
Mechanism· G2P
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GeneReview available — EFEMP2
Authoritative clinical overview · Recommended first read
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Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.60LOEUF
pLI 0.008
Z-score 3.03
OE 0.33 (0.200.60)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.98Z-score
OE missense 0.84 (0.750.93)
236 obs / 282.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.33 (0.200.60)
00.351.4
Missense OE?0.84 (0.750.93)
00.61.4
Synonymous OE?1.20
01.21.6
LoF obs/exp: 8 / 24.0Missense obs/exp: 236 / 282.1Syn Z: -1.70

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

EFEMP2 · 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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