KIF26A

Chr 14AR

kinesin family member 26A

Also known as: CDCBM11

KIF26A encodes an atypical kinesin that binds microtubules and regulates cerebral cortical development by controlling neurite growth and radial migration of cortical excitatory neurons. Biallelic mutations cause autosomal recessive complex cortical dysplasia with other brain malformations, affecting brain development and structure. The gene is highly constrained against loss-of-function variants (LOEUF 0.47), indicating strong evolutionary pressure to maintain protein function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
DNmechanismARLOEUF 0.471 OMIM phenotype
Clinical SummaryKIF26A
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Gene-Disease Validity (ClinGen)
complex cortical dysplasia with other brain malformations · ARStrong

Strong evidence — appropriate for clinical testing

Population Constraint (gnomAD)
Constrained for loss-of-function variants (OE-LoF 0.31) despite low pLI — interpret in context.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.47LOEUF
pLI 0.001
Z-score 4.62
OE 0.31 (0.210.47)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
0.09Z-score
OE missense 0.99 (0.941.04)
1119 obs / 1127.4 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.31 (0.210.47)
00.351.4
Missense OE0.99 (0.941.04)
00.61.4
Synonymous OE1.14
01.21.6
LoF obs/exp: 16 / 51.9Missense obs/exp: 1119 / 1127.4Syn Z: -2.46
DN
0.6647th %ile
GOF
0.6052th %ile
LOF
0.4726th %ile

The highest-scoring mechanism for this gene is dominant-negative.

DNprediction 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

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KIF26A · 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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