TDP1 Chromosome 14

Tyrosyl-DNA phosphodiesterase 1
9 variants 9 Health Risk

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What This Gene Does
The protein encoded by this gene is involved in repairing stalled topoisomerase I-DNA complexes by catalyzing the hydrolysis of the phosphodiester bond between the tyrosine residue of topoisomerase I and the 3-prime phosphate of DNA. This protein may also remove glycolate from single-stranded DNA containing 3-prime phosphoglycolate, suggesting a role in repair of free-radical mediated DNA double-strand breaks. This gene is a member of the phospholipase D family and contains two PLD phosphodiesterase domains. Mutations in this gene are associated with the disease spinocerebellar ataxia with axonal neuropathy (SCAN1). [provided by RefSeq, Aug 2016]
Associated Conditions (3)
Spinocerebellar ataxia
autosomal recessive
with axonal neuropathy 1
Key Variants
All Variants (9)
RSID Category Clinical Significance Conditions
RS200900192 Health Risk Conflicting classifications of pathogenicity
RS75808917 Health Risk Conflicting classifications of pathogenicity
RS769278668 Health Risk Conflicting classifications of pathogenicity
RS779666160 Health Risk Conflicting classifications of pathogenicity Spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 1
RS1376503364 Health Risk Likely pathogenic
RS1566929134 Health Risk Likely pathogenic
RS1892596331 Health Risk Likely pathogenic
RS370121773 Health Risk Likely pathogenic Spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 1
RS119467003 Health Risk Pathogenic Spinocerebellar ataxia, autosomal recessive, with axonal neuropathy 1
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