WT1 Alternative Splicing: Role of Its Isoforms in Neuroblastoma

Wilms tumor 1 ( WT1 ), a tumor suppressor gene, was originally identified in the homonymous renal neoplasm but is also involved in other cancers. Its function is still unclear, since it acts both as a pro- and an anti-oncogene. At least 14 WT1 transcriptional variants have been described; yet most i...

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Bibliographic Details
Published in:Journal of molecular neuroscience 2017-06, Vol.62 (2), p.131-141
Main Authors: Rasà, Daniela Maria, D’Amico, Agata Grazia, Maugeri, Grazia, Cavallaro, Sebastiano, D’Agata, Velia
Format: Article
Language:English
Subjects:
Alternative Splicing
Animals
Biomedical and Life Sciences
Biomedicine
Brain Neoplasms - genetics
Brain Neoplasms - pathology
Cancer
Cell Biology
Genes
Humans
Isoforms
Kidney cancer
Kidneys
Markers
Neuroblastoma
Neuroblastoma - genetics
Neuroblastoma - pathology
Neurochemistry
Neurology
Neurosciences
Protein Isoforms - chemistry
Protein Isoforms - genetics
Protein Isoforms - metabolism
Proteomics
Reviews
Transcription
Tumor markers
Tumor suppressor genes
WT1 protein
WT1 Proteins - chemistry
WT1 Proteins - genetics
WT1 Proteins - metabolism
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Description
Summary:Wilms tumor 1 ( WT1 ), a tumor suppressor gene, was originally identified in the homonymous renal neoplasm but is also involved in other cancers. Its function is still unclear, since it acts both as a pro- and an anti-oncogene. At least 14 WT1 transcriptional variants have been described; yet most investigations have focused on a small number of isoforms. We describe their structural features and review the evidence of their involvement in cancer with emphasis on neuroblastoma. In future, full characterization of all WT1 isoforms is expected to identify new molecular tumor markers and/or therapeutic targets.
ISSN:0895-8696
1559-1166
DOI:10.1007/s12031-017-0930-0