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10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
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ZNF141
zinc finger protein 141
Chromosome 4 Β· 4p16.3
NCBI Gene: 7700Ensembl: ENSG00000131127.14HGNC: HGNC:12926UniProt: D6RIY0
13PubMed Papers
21Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
limb morphogenesisnegative regulation of transcription by RNA polymerase IInucleusregulation of DNA-templated transcriptionpolydactyly, postaxial, type A6postaxial polydactyly type ABartholin gland diseasetelomere syndrome
✦AI Summary

ZNF141 is a C2-H2/KRAB zinc finger transcription factor located on chromosome 4.3 that functions as a transcriptional repressor involved in limb development 1. The protein contains DNA-binding domains that enable sequence-specific binding to RNA polymerase II cis-regulatory regions, allowing regulation of transcription and anatomical structure morphogenesis 2. ZNF141 mutations cause non-syndromic postaxial polydactyly, a common inherited limb abnormality characterized by supernumerary digits. Both autosomal recessive and dominant inheritance patterns have been documented, with identified mutations including missense variants (c.1420C>T; p.Thr474Ile) that disrupt normal limb anterior-posterior axis patterning 3245. ZNF141 is one of seven genes implicated in postaxial polydactyly pathogenesis 4. Beyond developmental roles, ZNF141 demonstrates clinical significance in malignancy. Expression analysis in soft tissue sarcomas identified ZNF141 as a component of prognostic models for overall and progression-free survival, with functional studies demonstrating that ZNF141 promotes sarcoma cell proliferation and viability 6. Additionally, ZNF141 shows differential expression in extranodal NK/T-cell lymphoma, where it is upregulated in affected patients 7. These findings suggest ZNF141 functions as both a developmental regulator and potential oncogenic factor.

Sources cited
1
ZNF141 is a C2-H2/KRAB zinc finger gene mapped to chromosome 4p16.3; functions as transcriptional regulator
PMID: 8268908
2
ZNF141 is a disease-causing gene for non-syndromic polydactyly with autosomal dominant inheritance
PMID: 26515020
3
ZNF141 missense mutation (c.1420C>T; p.Thr474Ile) causes autosomal recessive postaxial polydactyly type A
PMID: 23160277
4
ZNF141 is one of seven genes reported to cause non-syndromic postaxial polydactyly
PMID: 36071556
5
ZNF141 is among six genes identified causing non-syndromic polydactyly with autosomal dominant inheritance and variable penetrance
PMID: 30459804
6
ZNF141 promotes proliferation and viability of soft tissue sarcoma cells; included in prognostic models for overall and progression-free survival
PMID: 37100454
7
ZNF141 is upregulated in extranodal NK/T-cell lymphoma patients and identified as differential gene marker
PMID: 34872521
Disease Associationsβ“˜21
polydactyly, postaxial, type A6Open Targets
0.50Moderate
postaxial polydactyly type AOpen Targets
0.37Weak
Bartholin gland diseaseOpen Targets
0.04Suggestive
telomere syndromeOpen Targets
0.03Suggestive
osteosarcomaOpen Targets
0.02Suggestive
colorectal carcinomaOpen Targets
0.01Suggestive
adrenal cortex carcinomaOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
polydactylyOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.00Suggestive
Wolf-Hirschhorn syndromeOpen Targets
0.00Suggestive
extranodal nasal NK/T cell lymphomaOpen Targets
0.00Suggestive
adenocarcinomaOpen Targets
0.00Suggestive
developmental and epileptic encephalopathyOpen Targets
0.00Suggestive
Digestive System Mixed Adenoneuroendocrine CarcinomaOpen Targets
0.00Suggestive
meningiomaOpen Targets
0.00Suggestive
pulmonary alveolar proteinosisOpen Targets
0.00Suggestive
gastric cancerOpen Targets
0.00Suggestive
hepatocellular carcinomaOpen Targets
0.00Suggestive
ovarian cancerOpen Targets
0.00Suggestive
Polydactyly, postaxial A6UniProt
Pathogenic Variants1
NM_003441.4(ZNF141):c.1421C>T (p.Thr474Ile)Pathogenic
Polydactyly, postaxial, type A6
β˜†β˜†β˜†β˜†2013β†’ Residue 474
View on ClinVar β†—
Related Genes
IQCEProtein interaction57%GCM1Shared pathway50%GLIS3Shared pathway33%CCDC124Shared pathway33%ZNF768Shared pathway33%GMEB2Shared pathway33%
Tissue Expression6 tissues
Ovary
100%
Liver
42%
Bone Marrow
41%
Heart
40%
Lung
37%
Brain
26%
Gene Interaction Network
Click a node to explore
ZNF141IQCEGCM1GLIS3CCDC124ZNF768GMEB2
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted Β· UniProt Q15928
View on AlphaFold β†—
Constraintβ“˜
LOEUFβ“˜
1.12LoF Tolerant
pLIβ“˜
0.05Tolerant
Observed/Expected LoF0.57 [0.31–1.12]
RankingsWhere ZNF141 stands among ~20K protein-coding genes
  • #16,359of 20,598
    Most Researched13
  • #5,459of 5,498
    Most Pathogenic Variants1
  • #11,595of 17,882
    Most Constrained (LOEUF)1.12
Genes detectedZNF141
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Development and validation of novel prognostic models for zinc finger proteins-related genes in soft tissue sarcoma.
PMID: 37100454
Aging (Albany NY) Β· 2023
1.00
2
Advances in the molecular genetics of non-syndromic polydactyly.
PMID: 26515020
Expert Rev Mol Med Β· 2015
0.90
3
Genetic overview of postaxial polydactyly: Updated classification.
PMID: 36071556
Clin Genet Β· 2023
0.80
4
A zinc-finger gene ZNF141 mapping at 4p16.3/D4S90 is a candidate gene for the Wolf-Hirschhorn (4p-) syndrome.
PMID: 8268908
Hum Mol Genet Β· 1993
0.70
5
The identification of gene signatures in patients with extranodal NK/T-cell lymphoma from a pair of twins.
PMID: 34872521
BMC Cancer Β· 2021
0.60