TARDBP

Chr 1

TAR DNA binding protein

Also known as: ALS10, TDP-43

HIV-1, the causative agent of acquired immunodeficiency syndrome (AIDS), contains an RNA genome that produces a chromosomally integrated DNA during the replicative cycle. Activation of HIV-1 gene expression by the transactivator Tat is dependent on an RNA regulatory element (TAR) located downstream of the transcription initiation site. The protein encoded by this gene is a transcriptional repressor that binds to chromosomally integrated TAR DNA and represses HIV-1 transcription. In addition, this protein regulates alternate splicing of the CFTR gene. A similar pseudogene is present on chromosome 20. [provided by RefSeq, Jul 2008]

GeneReviewsResearchGenerating clinical summary…

Primary Disease Associations & Inheritance

UniProtAmyotrophic lateral sclerosis 10

Clinical highlights

Gene-disease validity (ClinGen)
amyotrophic lateral sclerosis type 10 · ADDefinitivesufficient evidence for diagnostic panels
Interpreting a novel variant
This gene is strongly intolerant of loss-of-function variation in the population, so LoF variants warrant close attention. No curated mechanism annotation is available — see the mechanism card for the computational prediction and its caveats.Based on population constraint only.
4
Active trials
121
Pubs (1 yr)
P/LP submissions
P/LP missense
0.28
LOEUF· LoF intol.
Multiple*
Mechanism· predicted
📖
GeneReview available — TARDBP
Authoritative clinical overview · Recommended first read
Open GeneReview ↗
Some data sources returned errors (1)

omim: Error: OMIM fetch failed: 429

Population Genetics & Constraint

gnomAD v4 — loss-of-function & missense intolerance

Dual constrained — LoF & missense intolerant
LoF Constraint?
0.28LOEUF
pLI 0.985
Z-score 3.62
OE 0.06 (0.020.28)
Highly constrained

Highly LoF-intolerant (top ~10% of genes)

Missense Constraint?
3.71Z-score
OE missense 0.32 (0.260.39)
74 obs / 233.5 exp
Constrained

Highly missense-constrained (top ~0.1%)

Observed / Expected Ratios?
LoF OE?0.06 (0.020.28)
00.351.4
Missense OE?0.32 (0.260.39)
00.61.4
Synonymous OE?1.10
01.21.6
LoF obs/exp: 1 / 17.2Missense obs/exp: 74 / 233.5Syn Z: -0.75

ClinVar Variant Classifications

0 submitted variants in ClinVar

Protein Context — Lollipop Plot

TARDBP · protein map & ClinVar variants

Showing all ClinVar variants across the protein. Search a specific variant to highlight its position.