SLCO1B1

Chr 12Digenic recessive

solute carrier organic anion transporter family member 1B1

Also known as: HBLRR, LST-1, OATP-C, OATP1B1, OATP2, OATPC, SLC21A6

The protein functions as a liver-specific organic anion transporter that mediates sodium-independent uptake of endogenous compounds including bilirubin, bile acids, and hormones, as well as drugs like statins. Mutations cause Rotor type hyperbilirubinemia through digenic recessive inheritance, requiring variants in both this gene and another gene for disease manifestation. This gene is not constrained against loss-of-function variants, consistent with its recessive inheritance pattern.

Summary from RefSeq, OMIM, UniProt
Research Assistant →

Primary Disease Associations & Inheritance

Hyperbilirubinemia, Rotor type, digenicMIM #237450
Digenic recessive
7
Active trials
106
Pubs (1 yr)
66
P/LP submissions
0%
P/LP missense
1.35
LOEUF
Multiple*
Mechanism· predicted
Clinical SummarySLCO1B1
Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
📋
ClinVar Variants
50 unique Pathogenic / Likely Pathogenic· 127 VUS of 266 total submissions
💊
Clinical Trials
7 active or recruiting trials — potential therapeutic options may be available
📖
GeneReview available — SLCO1B1
Authoritative clinical overview · Recommended first read
Open GeneReview ↗

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
1.35LOEUF
pLI 0.000
Z-score -0.04
OE 1.01 (0.761.35)
Tolerant

Highly tolerant — LoF variants common in population

Missense Constraint
-0.90Z-score
OE missense 1.13 (1.041.23)
404 obs / 356.3 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE1.01 (0.761.35)
00.351.4
Missense OE1.13 (1.041.23)
00.61.4
Synonymous OE0.96
01.21.6
LoF obs/exp: 32 / 31.7Missense obs/exp: 404 / 356.3Syn Z: 0.32
DN
0.7036th %ile
GOF
0.6345th %ile
LOF
0.3065th %ile

This gene has evidence for multiple mechanisms of pathogenicity (dominant-negative and gain-of-function). Both the Badonyi & Marsh prediction and the broader genomic evidence point to dominant-negative as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

DNprediction above median
GOFprediction above median

Note: In-silico variant effect predictors (SIFT, PolyPhen, REVEL, CADD) may underestimate pathogenicity of missense variants in genes with GOF or DN mechanisms. Consider functional evidence and clinical context.

Predictions from Badonyi M, Marsh JA. PLoS ONE. 2024;19(8):e0307312.

ClinVar Variant Classifications

266 submitted variants in ClinVar

Classification Summary

Pathogenic42
Likely Pathogenic8
VUS127
Likely Benign27
Benign52
Conflicting4
42
Pathogenic
8
Likely Pathogenic
127
VUS
27
Likely Benign
52
Benign
4
Conflicting

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
0
0
42
0
42
Likely Pathogenic
4
0
4
0
8
VUS
9
84
30
4
127
Likely Benign
0
6
11
10
27
Benign
0
5
40
7
52
Conflicting
4
Total139512721260

LoF = frameshift, stop gained/lost, canonical splice · Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

SLCO1B1 · 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

Clinical Literature
Open Research Assistant →
Full-Text Mentions
NLP-detected gene mentions in article bodies · via PubTator3
PubTator3
Top 5 full-text resultsSearch PubTator3 ↗
Key Publications
Landmark & review papers · by relevance
PubMed
Top 5 results · since 2015Search PubMed ↗