KATNAL1

Chr 13

katanin catalytic subunit A1 like 1

The protein is an ATP-dependent microtubule-severing enzyme that regulates microtubule dynamics, particularly in Sertoli cells where it is essential for spermiogenesis and male fertility. Mutations cause autosomal recessive male infertility due to multiple morphological abnormalities of the sperm flagella (MMAF), resulting in asthenozoospermia and oligozoospermia. The gene is highly constrained against loss-of-function variants, indicating its critical cellular role.

OMIMResearchSummary from RefSeq, UniProt
DNmechanismLOEUF 0.44
Clinical SummaryKATNAL1
Population Constraint (gnomAD)
Moderately constrained gene (pLI 0.53) — some intolerance to loss-of-function variants.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Moderate LoF intolerance
LoF Constraint
0.44LOEUF
pLI 0.532
Z-score 3.59
OE 0.21 (0.110.44)
Moderately constrained

More LoF-intolerant than ~75% of genes

Missense Constraint
1.60Z-score
OE missense 0.72 (0.640.81)
190 obs / 263.2 exp
Tolerant

Mild missense constraint

Observed / Expected Ratios
LoF OE0.21 (0.110.44)
00.351.4
Missense OE0.72 (0.640.81)
00.61.4
Synonymous OE0.93
01.21.6
LoF obs/exp: 5 / 23.9Missense obs/exp: 190 / 263.2Syn Z: 0.55
DN
0.6743th %ile
GOF
0.5660th %ile
LOF
0.4135th %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

KATNAL1 · 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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