UNIPROT #

Q9UMX1

PROTEIN NAME

SUFU

SYNONYMS

OTTHUMP00000020374 antibody; OTTHUMP00000020377 antibody; OTTHUMP00000020379 antibody; PRO1280 antibody; SU FU antibody; SU(F)U antibody; Su(fu) antibody; SUFU antibody; SUFU negative regulator of hedgehog signaling antibody; SUFU_HUMAN antibody; SUFUH antibody; SUFUXL antibody; Suppressor of fused homolog (Drosophila) antibody; Suppressor of fused homolog antibody

SEQUENCE SIMILARITIES

Belongs to the SUFU family.

TISSUE SPECIFICITY

Ubiquitous in adult tissues. Detected in osteoblasts of the perichondrium in the developing limb of 12-week old embryos. Isoform 1 is detected in fetal brain, lung, kidney and testis. Isoform 2 is detected in fetal testis, and at much lower levels in fetal brain, lung and kidney.

POST-TRANSLATIONAL MODIFICATION

Polyubiquitinated at Lys-257 by the SCF(FBXL17) complex, leading to its subsequent degradation and allowing the release of GLI1 for proper hedgehog/smoothened signal transduction. Ubiquitination is impaired by phosphorylation at Ser-342, Ser-346, Ser-352 and Thr-353.; Phosphorylation at Ser-342, Ser-346, Ser-352 and Thr-353 prevents ubiquitination by the SCF(FBXL17) complex.

SUBCELLULAR LOCATION

Cytoplasm. Nucleus.

FUNCTION

Su(fu) (for Suppressor-of-Fused) is a key negative regulator in the vertebrate Hedgehog signaling pathway. Su(fu) interacts with genes encoding proteins in this signal transduction pathway. In Drosophila, Intracellular transduction of the Hedgehog pathway involves the release of a large complex containing Su(fu). Su(fu) inhibits the activity of the transcription factor Gli1 and interacts with Gli2, Gli3 and the serine/threonine kinase Fused. Su(fu) is widely expressed in adult and embryonic tissues with higher expression in tissues patterned by hegdehog signaling. The Su(fu) gene locus maps to a region that is deleted in glioblastomas, prostate cancer, malignant melanoma and endometrial cancer.