zinc finger transcription factor Trps1; tricho-rhino-phalangeal syndrome type I protein; zinc finger protein GC79 (TRPS1)

Function

• transcriptional repressor
• binds specifically to GATA sequences & represses expression of GATA-regulated genes at selected sites & stages in vertebrate development
• sumoylation in the repressor domain inhibits the transcriptional repression activity
• sumoylation on Lys-1201 is the major site
• appears to be sumoylated on multiple sites
• interacts with RNF4
• regulates TRPS1 repressor activity

Structure

• contains 7 C2H2-type Zn+2 fingers
• contains 1 GATA-type Zn+2 finger

Compartment

nucleus

Alternative splicing

named isoforms=3

Expression

• ubiquitously expressed in the adult
• found in fetal brain, lung, kidney, liver, spleen & thymus
• more highly expressed in androgen-dependent than in androgen-independent prostate cancer cells

Pathology

• defects in TRPS1 are the cause of
  • tricho-rhino-phalangeal syndrome type 1
  • tricho-rhino-phalangeal syndrome type 3
• chromosomal aberration involving TRPS1 is a cause of tricho-rhino-phalangeal syndrome type 2

More general terms

• nuclear protein
• C2H2 type zinc finger protein
• phosphoprotein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=7227
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:7227
• OMIM: https://mirror.omim.org/entry/150230
• OMIM: https://mirror.omim.org/entry/190350
• OMIM: https://mirror.omim.org/entry/190351
• OMIM: https://mirror.omim.org/entry/604386
• UniProt: http://www.uniprot.org/uniprot/Q9UHF7.html