ABCC6

Chr 16

ATP binding cassette subfamily C member 6

Also known as: ABC34, ARA, EST349056, GACI2, MLP1, MOAT-E, MOATE, MRP6

The 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 intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). The encoded protein, a member of the MRP subfamily, is involved in multi-drug resistance. Mutations in this gene cause pseudoxanthoma elasticum. Alternatively spliced transcript variants that encode different proteins have been described for this gene. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtPseudoxanthoma elasticum
UniProtArterial calcification of infancy, generalized, 2

Clinical highlights

Gene-disease validity (ClinGen)
inherited pseudoxanthoma elasticum · SDDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
Loss of function is the curated mechanism (Gene2Phenotype), though the gene is not strongly LoF-constrained in the population — weigh truncating variants against that tolerance.Curated gene-level mechanism — a prior for triage, not a per-variant call.
4
Active trials
74
Pubs (1 yr)
P/LP submissions
P/LP missense
1.03
LOEUF
LOF
Mechanism· G2P
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GeneReview available — ABCC6
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?
1.03LOEUF
pLI 0.000
Z-score 1.36
OE 0.83 (0.671.03)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint?
-1.09Z-score
OE missense 1.11 (1.051.17)
926 obs / 837.4 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios?
LoF OE?0.83 (0.671.03)
00.351.4
Missense OE?1.11 (1.051.17)
00.61.4
Synonymous OE?1.07
01.21.6
LoF obs/exp: 60 / 72.5Missense obs/exp: 926 / 837.4Syn Z: -1.09

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

ABCC6 · protein map & ClinVar variants

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