suppressor of fused homolog; SUFUH (SUFU)

Function

• negative regulator hedgehog signaling
• down-regulates GLI1-mediated transactivation of target genes.
• component of corepressor complex that acts on DNA-bound GLI1
• may link GLI1 to BTRC, thus target GLI1 to degradation by the proteasome
• sequesters GLI1, GLI2, GLI3 in cytoplasm
  • effect is overcome by binding of STK36 to both SUFU & GLI protein
• negatively regulates beta-catenin signaling
• part of a DNA-bound corepressor complex containing SAP18, GLI1, SIN3
• binds BTRC, GLI2, GLI3, SAP18, STK36.
• binds both free & DNA-bound GLI1
• part of a DNA-bound corepressor complex
• interacts with KIF7
• interacts with GLI3FL
  • interaction regulates formation of either repressor or activator forms of GLI3
  • association with GLI3FL is regulated by Hh signaling & dissociation of the SUFU-GLI3
  • interaction requires the presence of the ciliary motor KIF3A
• interacts with ULK3; inactivating the protein kinase activity of ULK3

Structure

belongs to the SUFU family

Compartment

cytoplasm. nucleus

Alternative splicing

named isoforms=3

Expression

• 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 & testis
• isoform 2 is expressed in fetal testis > fetal brain, lung & kidney

Pathology

• defects in SUFU are a cause of medulloblastoma

More general terms

• serine/threonine kinase

Additional terms

• serine/threonine protein kinase 36; fused Drosophila homolog of FU; fused homolog (STK36 KIAA1278)

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=51684
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:51684
• OMIM: https://mirror.omim.org/entry/155255
• OMIM: https://mirror.omim.org/entry/607035
• UniProt: http://www.uniprot.org/uniprot/Q9UMX1.html