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transcription factor PML; tripartite motif-containing protein 19; RING finger protein 71 (PML, MYL, RNF71, TRIM19)

Function: - probable transcription factor - may play role in recruitment of ELF4 into PML nuclear bodies - interacts with SIRT1, TOPBP1, TRIM27 & TRIM69 - interacts with the C-terminus of ELF4. - interacts with Lassa virus Z protein & rabies virus phosphoprotein (putative) - ubiquitinated; mediated by SIAH1 or SIAH2 & leading to its subsequent proteasomal degradation - sumoylation on all three sites is required for nuclear body formation - sumoylation on Lys-160 is a prerequisite for sumoylation on Lys-65 - the PML-RARA fusion protein is not sumoylated Structure: - contains 2 B box-type Zn+2 fingers contains 1 RING-type Zn+2 finger Compartment: - nucleus, nucleoplasm, cytoplasm - sumoylated forms localize to the PML nuclear bodies - the B1 box & the RING finger are also required for this nuclear localization - isoforms lacking a nuclear localization signal are cytoplasmic Alternative splicing: - named isoforms=5 - additional isoforms seem to exist Pathology: - chromosomal translocation t(15;17)(q21;q21) involving PML with RARA may be a cause of acute promyelocytic leukemia; the PML breakpoints (type A & type B) lie on either side of an alternatively spliced exon - autoantibody ? (see Laboratory) Laboratory: - PML antibody in serum [3]

Related

AML-M3; acute promyelocytic leukemia PML gene

Specific

transcription factor PML-1 transcription factor PML-2 transcription factor PML-3 transcription factor PML-3B transcription factor PML-X or My1 protein

General

DNA-binding protein proto oncogene protein ring finger protein transcription factor (TF)

Properties

SIZE: entity length = 882 aa MW = 98 kD COMPARTMENT: cytoplasm cell nucleus MOTIF: proline-rich region SITE: 3-46 MOTIF: proline residue (SEVERAL) Ser phosphorylation site {S8} RING-finger {57-92} MOTIF: Zn+2-binding site SITE: 57-57 Zn+2-binding site SITE: 60-60 Zn+2-binding site SITE: 72-72 Zn+2-binding site SITE: 74-74 Zn+2-binding site SITE: 77-77 Zn+2-binding site SITE: 80-80 Zn+2-binding site SITE: 88-88 Zn+2-binding site SITE: 91-91 EFFECTOR-BOUND: Zn+2 FOR-BINDING-OF: DNA motif zinc finger B box SITE: 124-166 EFFECTOR-BOUND: Zn+2 zinc finger B box SITE: 183-236 EFFECTOR-BOUND: Zn+2 coiled coil {228-253} Breakpoint {394-395} Ser phosphorylation site {S403} nuclear translocation signal {476-490} Ser phosphorylation site {S504} Ser phosphorylation site {S505} Ser phosphorylation site {S512} Ser phosphorylation site {S518} Ser phosphorylation site {S527} Ser phosphorylation site {S530} Ser phosphorylation site {S535} Breakpoint {552-553} Ser phosphorylation site {S560} Ser phosphorylation site {S561} Ser phosphorylation site {S562} Ser phosphorylation site {S565}

Database Correlations

OMIM 102578 UniProt P29590 PFAM correlations Entrez Gene 5371 Kegg hsa:5371

References

  1. UniProt :accession P29590
  2. Atlas of genetics & cytogenetics in oncology & haematology http://atlasgeneticsoncology.org/genes/PMLID41.html
  3. Loinc

Component-of

PML NB complex