Wilms Tumor (WT) is a 55 kDa protein thought to play a major role in various cancers and developmental disorders, commonly kidney tumors and glomerular diseases. WT is a zinc finger transcription factor that is mutated in Wilms tumors and highly expressed in a wide variety of other malignancies (1). WT1 consists of two major isoforms; WT1 (-KTS), a transcription factor, and WT1 (+KTS) (2). Mutations in the zinc finger regions of WT are detected in nearly all patients of Denys-Drash syndrome and in some patients with isolated diffuse mesangial sclerosis. Also, mutations leading to the loss of the transcription factor isoform have been observed in all patients with Frasier syndrome.
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