WDR73

WDR73
Identifiers
AliasesWDR73, GAMOS, HSPC264, WD repeat domain 73, GAMOS1
External IDsOMIM: 616144; MGI: 1919218; GeneCards: WDR73
Orthologs
DatabasesNCBI: entry; OMA: entry
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

NM_032856

NM_028026

RefSeq (protein)

NP_116245
NP_116245.2

NP_082302

Location (UCSC)Chr 15: 84.64 – 84.65 MbChr 7: 80.54 – 80.55 Mb
PubMed search
Wikidata
View/Edit HumanView/Edit Mouse

The integrator complex assembly factor WDR73 is a protein that in humans is encoded by the gene WDR73. This protein participates in the assembly of the multi-protein complex called the integrator complex, which regulates RNA polymerase II.

Function

The protein WDR73 interacts with two subunits of the integrator complex, INTS9 and INTS11, and helps them bind together to form a heterodimer through stabilization. This blocks the active site of INTS11. BRAT1 then comes and takes WDR73's place at the active site, releasing it, and subsequently importing INTS9 and INTS11 into the cell nucleus.

Structure

Most of this protein consists of 6 WD40 repeat domains. This type of domain is common in scaffold proteins and often participates in protein-protein or protein-RNA interactions.

Medical significance

Pathogenic mutations in WDR73 is associated with a neurodegenerative disorder called type 1 Galloway-Mowat syndrome (GAMOS1). Symptoms can include microcephaly, various forms of brain atrophy, and movement disorders. It is inherited in an autosomal recessive manner.

References

  1. 1 2 3 GRCh38: Ensembl release 89: ENSG00000177082 – Ensembl, May 2017
  2. 1 2 3 GRCm38: Ensembl release 89: ENSMUSG00000025722 – Ensembl, May 2017
  3. ↑ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ↑ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. 1 2 3 4 5 6 "Integrator complex assembly factor WDR73". uniprot.org. UniProt consortium. Retrieved 2026-11-01.
  6. ↑ Schapira M, Tyers M, Torrent M, Arrowsmith CH (November 2017). "WD40 repeat domain proteins: a novel target class?" (PDF). Nature reviews Drug discovery. 16 (11): 773–86. doi:10.1038/nrd.2017.179.
  7. 1 2 3 McKusick VA, Kniffin CL (2025-09-22). "GALLOWAY-MOWAT SYNDROME 1; GAMOS1". OMIM. Johns Hopkins university. Retrieved 2026-11-01.
  8. ↑ Sabath K, Jonas S (2022-12-01). "Take a break: Transcription regulation and RNA processing by the Integrator complex". Current Opinion in Structural Biology. 77: 102443. doi:10.1016/j.sbi.2022.102443.{{cite journal}}: CS1 maint: article number as page number (link)