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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CTDSP1
CTD small phosphatase 1
Chromosome 2 · 2q35
NCBI Gene: 58190Ensembl: ENSG00000144579.10HGNC: HGNC:21614UniProt: Q9GZU7
50PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleoplasmextracellular exosomeRNA polymerase II CTD heptapeptide repeat phosphatase activityprotein bindingAlzheimer diseaseParkinson diseaselysosomal storage diseasemultiple sclerosis
✦AI Summary

CTDSP1 (CTD small phosphatase 1) is a nuclear phosphatase that preferentially dephosphorylates serine-5 residues within the heptapeptide repeats of RNA polymerase II's C-terminal domain (CTD), thereby negatively regulating transcription during the transition from initiation to elongation 1. CTDSP1 functions as a component of the REST repressor complex, silencing neuronal genes in non-neuronal cells through RE-1 element-mediated mechanisms 1. Beyond transcriptional regulation, CTDSP1 dephosphorylates multiple oncogenic substrates including AKT1, Ras family proteins, TWIST1, c-MYC, and regulates the retinoblastoma protein (pRb) at the G1/S cell-cycle checkpoint 2. CTDSP1 exhibits prominent tumor suppressor activity in clear cell renal cell carcinoma (ccRCC), where CTDSP1 overexpression significantly inhibits cell growth and associates with improved patient survival 3. In lung cancer, CTDSP1 suppresses progression and metastasis via AhR-ARNT signaling and exosomal miR-26b transfer 4. Conversely, CTDSP1 inhibition by rabeprazole promotes irinotecan resistance in colorectal cancer by facilitating topoisomerase I degradation 5. CTDSP1 knockdown promotes peripheral nerve regeneration by destabilizing REST, enhancing neurotrophic factor expression 6. Notably, human CTDSP1 functionally conserves with yeast HAD phosphatases in regulating TORC1-autophagy signaling under amino acid limitation 7. These diverse functions underscore CTDSP1's multifaceted role in transcriptional regulation, cell-cycle control, and therapeutic resistance.

Sources cited
1
CTDSP1 dephosphorylates serine-5 of RNAP II CTD heptad repeats and participates in REST complex-mediated neuronal gene silencing
PMID: 32397221
2
CTDSP1 regulates multiple substrates including AKT1, Ras proteins, TWIST1, c-MYC, and pRb at G1/S checkpoint
PMID: 34432772
3
CTDSP1 exhibits tumor suppressor activity in ccRCC with reduced expression correlating with poor survival
PMID: 37629167
4
CTDSP1 suppresses lung cancer progression via AhR-ARNT signaling and exosomal miR-26b regulation
PMID: 34079082
5
CTDSP1 inhibition by rabeprazole promotes irinotecan resistance through topoisomerase I degradation
PMID: 32764831
6
CTDSP1 knockdown promotes peripheral nerve regeneration by destabilizing REST and enhancing neurotrophic factor expression
PMID: 34262056
7
Human CTDSP1 functionally replaces yeast HAD phosphatases in regulating TORC1 and autophagy under leucine limitation
PMID: 40103213
Disease Associationsⓘ20
Alzheimer diseaseOpen Targets
0.37Weak
lysosomal storage diseaseOpen Targets
0.36Weak
multiple sclerosisOpen Targets
0.36Weak
neurodegenerative diseaseOpen Targets
0.36Weak
Parkinson diseaseOpen Targets
0.36Weak
Precordial painOpen Targets
0.28Weak
neuroinflammatory disorderOpen Targets
0.26Weak
nonpapillary renal cell carcinomaOpen Targets
0.05Suggestive
attention deficit hyperactivity disorderOpen Targets
0.04Suggestive
hereditary attention deficit-hyperactivity disorderOpen Targets
0.04Suggestive
neoplasmOpen Targets
0.04Suggestive
esophageal squamous cell carcinomaOpen Targets
0.03Suggestive
portal hypertensionOpen Targets
0.03Suggestive
non-small cell lung carcinomaOpen Targets
0.03Suggestive
squamous cell carcinomaOpen Targets
0.02Suggestive
AIDS dementiaOpen Targets
0.02Suggestive
cancerOpen Targets
0.02Suggestive
acute lymphoblastic leukemiaOpen Targets
0.02Suggestive
cardiac hypertrophyOpen Targets
0.01Suggestive
breast cancerOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PTPN4Shared pathway100%PTPN18Shared pathway100%DAPP1Shared pathway100%PPM1MShared pathway100%PTPN5Shared pathway100%RESTProtein interaction99%
Tissue Expression6 tissues
Lung
100%
Liver
99%
Ovary
87%
Heart
68%
Bone Marrow
38%
Brain
13%
Gene Interaction Network
Click a node to explore
CTDSP1PTPN4PTPN18DAPP1PPM1MPTPN5REST
PROTEIN STRUCTURE
Preparing viewer…
PDB2GHT · 1.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.69LoF Tolerant
pLIⓘ
0.09Tolerant
Observed/Expected LoF0.45 [0.30–0.69]
RankingsWhere CTDSP1 stands among ~20K protein-coding genes
  • #8,794of 20,598
    Most Researched50
  • #5,152of 17,882
    Most Constrained (LOEUF)0.69
Genes detectedCTDSP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Tumor Suppressor Properties of Small C-Terminal Domain Phosphatases in Clear Cell Renal Cell Carcinoma.
PMID: 37629167
Int J Mol Sci · 2023
1.00
2
Targeting the C-Terminal Domain Small Phosphatase 1.
PMID: 32397221
Life (Basel) · 2020
0.90
3
[SCP Phosphatases and Oncogenesis].
PMID: 34432772
Mol Biol (Mosk) · 2021
0.80
4
GPC5 suppresses lung cancer progression and metastasis via intracellular CTDSP1/AhR/ARNT signaling axis and extracellular exosome secretion.
PMID: 34079082
Oncogene · 2021
0.70
5
Porocarcinomas with PAK1/2/3 fusions: a series of 12 cases.
PMID: 38785043
Histopathology · 2024
0.60