ABCA3

Chr 16AR

ATP binding cassette subfamily A member 3

Also known as: ABC-C, ABC3, EST111653, LBM180, SMDP3

The membrane-associated protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intracellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ABC1 subfamily. Members of the ABC1 subfamily comprise the only major ABC subfamily found exclusively in multicellular eukaryotes. The full transporter encoded by this gene may be involved in development of resistance to xenobiotics and engulfment during programmed cell death. [provided by RefSeq, Jul 2008]

GeneReviewsOMIMResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

Surfactant metabolism dysfunction, pulmonary, 3MIM #610921
AR
UniProtPulmonary surfactant metabolism dysfunction 3

Clinical highlights

Gene-disease validity (ClinGen)
interstitial lung disease due to ABCA3 deficiency · ARDefinitivesufficient evidence for diagnostic panels
1
Active trials
54
Pubs (1 yr)
P/LP submissions
P/LP missense
0.50
LOEUF
Multiple*
Mechanism· predicted
📖
GeneReview available — ABCA3
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint?
0.50LOEUF
pLI 0.000
Z-score 5.02
OE 0.36 (0.260.50)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint?
0.32Z-score
OE missense 0.97 (0.921.02)
995 obs / 1024.1 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.36 (0.260.50)
00.351.4
Missense OE?0.97 (0.921.02)
00.61.4
Synonymous OE?1.16
01.21.6
LoF obs/exp: 26 / 72.0Missense obs/exp: 995 / 1024.1Syn Z: -2.75

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ABCA3 · protein map & ClinVar variants

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