BUB1

Chr 2AR

BUB1 mitotic checkpoint serine/threonine kinase

Also known as: BUB1A, BUB1L, MCPH30, hBUB1

This gene encodes a serine/threonine-protein kinase that play a central role in mitosis. The encoded protein functions in part by phosphorylating members of the mitotic checkpoint complex and activating the spindle checkpoint. This protein also plays a role in inhibiting the activation of the anaphase promoting complex/cyclosome. This protein may also function in the DNA damage response. Mutations in this gene have been associated with aneuploidy and several forms of cancer. Alternate splicing results in multiple transcript variants. [provided by RefSeq, Jul 2013]

OMIMResearchGenerating clinical summary…
DNmechanismARLOEUF 0.602 OMIM phenotypes
Clinical SummaryBUB1
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Gene-Disease Validity (ClinGen)
colorectal cancer · ADLimited

Limited evidence — not for standalone diagnostic reporting

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.
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Clinical Trials
1 active or recruiting trial — potential therapeutic options may be available

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint?
0.60LOEUF
pLI 0.000
Z-score 3.98
OE 0.43 (0.310.60)
Tolerant

Typical tolerance to LoF variation

Missense Constraint?
0.85Z-score
OE missense 0.90 (0.830.97)
500 obs / 556.3 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios?
LoF OE?0.43 (0.310.60)
00.351.4
Missense OE?0.90 (0.830.97)
00.61.4
Synonymous OE?0.96
01.21.6
LoF obs/exp: 24 / 56.2Missense obs/exp: 500 / 556.3Syn Z: 0.44
Curated Mechanism (G2P)Gene2Phenotype (DDG2P) ↗
moderateBUB1-related microcephaly and developmental disorderOTHERAR

This gene — mechanism propensity

Predictions shown for reference only — model trained on dominant genes, not applicable to AR conditions.

DN
0.7132th %ile
GOF
0.4282th %ile
LOF
0.3356th %ile

The Badonyi & Marsh prediction model was trained exclusively on dominant disease genes. Predictions are not reliable for genes with autosomal recessive inheritance and are shown at reduced opacity for reference only.

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

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

BUB1 · protein map & ClinVar variants

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