KATNIP

Chr 16AR

katanin interacting protein

Also known as: JBTS26, KIAA0556

This gene encodes a ciliary protein that localizes to the ciliary axoneme and tip, and may influence microtubule stability through interaction with the katanin complex. Mutations cause Joubert syndrome 26, an autosomal recessive ciliopathy characterized by distinctive mid-hindbrain and cerebellar malformations often accompanied by broader ciliopathy symptoms. Patient-derived fibroblasts show reduced ciliogenesis with abnormally long cilia, consistent with the protein's role in ciliary function.

OMIMResearchSummary from RefSeq, OMIM, UniProt
ARLOEUF 0.911 OMIM phenotype
Clinical SummaryKATNIP
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Gene-Disease Validity (ClinGen)
ciliopathy · ARDefinitive

Definitive — sufficient evidence for diagnostic panels

Population Constraint (gnomAD)
Low constraint (pLI 0.00) — loss-of-function variants are relatively tolerated in the population.

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Tolerant — LoF & missense variants common in population
LoF Constraint
0.91LOEUF
pLI 0.000
Z-score 2.23
OE 0.73 (0.590.91)
Tolerant

Typical tolerance to LoF variation

Missense Constraint
-0.11Z-score
OE missense 1.01 (0.961.06)
987 obs / 977.2 exp
Tolerant

Tolerant to missense variation

Observed / Expected Ratios
LoF OE0.73 (0.590.91)
00.351.4
Missense OE1.01 (0.961.06)
00.61.4
Synonymous OE1.01
01.21.6
LoF obs/exp: 57 / 78.3Missense obs/exp: 987 / 977.2Syn Z: -0.18

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

KATNIP · 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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Recent Gene-Specific Literature
Gene in title · MEDLINE · newest first
Europe PMC