KANTR

Chr X

KANTR integral membrane protein

Also known as: LINC01155, Spasm

This gene produces a long non-coding RNA involved in neurological development or function. Mutations cause autosomal recessive spastic tetraplegia with thin corpus callosum, presenting in early childhood with progressive spasticity, developmental delay, and brain structural abnormalities. The gene appears to be highly constrained against loss-of-function variants, suggesting intolerance to disruption.

OMIMResearchSummary from RefSeq
Multiplemechanism

Population Genetics & Constraint

Constraint data not available from gnomAD.

DN
0.81top 10%
GOF
0.93top 5%
LOF
0.3260th %ile

This gene has evidence for multiple mechanisms of pathogenicity (gain-of-function and dominant-negative). Both the Badonyi & Marsh prediction and the broader genomic evidence point to gain-of-function as the predominant mechanism. Different variants in this gene may act through different mechanisms — interpret in context of the specific variant.

GOFprediction above median
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

KANTR · 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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