BOLA2B

Chr 16

bolA family member 2B

BOLA2B encodes a cytosolic iron-sulfur cluster assembly factor that facilitates insertion of [2Fe-2S] clusters into cytosolic proteins, working together with the monothiol glutaredoxin GLRX3. This gene is located within a segmental duplication on chromosome 16 and is identical to BOLA2, but no specific human diseases have been established for BOLA2B mutations in the provided data. The inheritance pattern and clinical phenotypes associated with BOLA2B variants remain to be determined.

ResearchSummary from RefSeq, UniProt
Multiplemechanism
Clinical SummaryBOLA2B
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ClinVar Variants
90 unique Pathogenic / Likely Pathogenic· 6 VUS of 98 total submissions

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.7229th %ile
GOF
0.6735th %ile
LOF
0.2774th %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

98 submitted variants in ClinVar

Classification Summary

Pathogenic73
Likely Pathogenic17
VUS6
73
Pathogenic
17
Likely Pathogenic
6
VUS

Curated Variants Distribution

Classified variants from ClinVar · 5 ACMG categories· variant type breakdown unavailable

ClassificationLoFMissense + InframeNon-codingSynonymousTotal
Pathogenic
73
Likely Pathogenic
17
VUS
6
Likely Benign
0
Benign
0
Total96

Counts from ClinVar esearch · Updated hourly

View in ClinVar →

Protein Context — Lollipop Plot

BOLA2B · protein map & ClinVar variants

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

3D Protein StructureAlphaFold

Clinical Trials

Active and recruiting trials from ClinicalTrials.gov

No active trials found for this gene.

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Clinical Literature
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