Transcription factor required for development of the vascular component of the pronephric kidney, the glomus; may repress tubule-specific gene expression in the portion of the pronephros fated to form ...[+]

Transcription factor required for development of the vascular component of the pronephric kidney, the glomus; may repress tubule-specific gene expression in the portion of the pronephros fated to form the glomus. Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3'. Inhibits Wnt-signaling during embryonic development. Function may be isoform-specific: the isoform containing the KTS motif is less effective in inhibiting wnt signaling. [-]

             nephrotic syndrome
             aniridia
             WAGR syndrome
             malignant mesothelioma
             nephroblastoma
             Denys-Drash syndrome

          DENYS-DRASH SYNDROME; DDS
          FRASIER SYNDROME
          NEPHROTIC SYNDROME, TYPE 4; NPHS4
          WILMS TUMOR, ANIRIDIA, GENITOURINARY ANOMALIES, AND MENTAL RETARDATION SYNDROME;
          WILMS TUMOR 1; WT1
          MESOTHELIOMA, MALIGNANT; MESOM
          MEACHAM SYNDROME

ClinVar, ClinGen, MalaCards, KEGG Disease, DECIPHER, COSMIC, MARRVEL, GTR, DisGeNET, dbVAR, GHR, gnomAD