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lipoma-preferred partner (LIM domain-containing preferred translocation partner in lipoma, LPP)
Function:
1) structural role at sites of cell adhesion in maintaining cell shape & motility
2) role in signaling & activation of gene transcription (putative)
3) role in signal transduction from cell adhesion sites to nucleus (putative)
4) scaffold protein upon which protein complexes are assembled in cytoplasm & in nucleus (putative)
5) interacts with VASP, with PDZ domains of SCRIB & with ACTN1/ alpha-actinin
Structure:
- contains 3 LIM zinc-binding domains
Compartment:
- nucleus, cytoplasm, cell junction, cell membrane (adhesion sites)
- shuttles between cytoplasm & nucleus
- sites of cell adhesion, cell-to-cell contact & focal adhesion
- mainly nuclear when fused with HMGA2/HMGIC & MLL
Expression:
- expressed in a wide variety of tissues
- no or very low expression in brain & peripheral leukocytes
Pathology:
- chromosomal translocation t(3;12)(q27-q28;q13-q15) involving LPP with HMGA2 is associated with lipomas
- chromosomal translocation t(3;12)(q28;q14) involving LPP with HMGA2 is associated with parosteal lipoma
- chromosomal translocation t(3;12)(q27-q28;q14-q15) involving LPP with HMGA2 is detected in pulmonary chondroid hamartomas
- chromosomal translocation t(3;11)(q28;q23) involving LPP with MLL is associated with acute monoblastic leukemia
General
adhesion protein
phosphoprotein
Properties
SIZE: MW = 66 kD
entity length = 612 aa
COMPARTMENT: cytoplasm
cell nucleus
MOTIF: proline-rich region
SITE: 41-370
MOTIF: proline residue (SEVERAL)
Ser phosphorylation site {S116}
Ser phosphorylation site {S126}
Tyr phosphorylation site {Y297}
Tyr phosphorylation site {Y300}
Tyr phosphorylation site {Y301}
Tyr phosphorylation site {Y317}
Breakpoint {371-372}
LIM domain {414-473}
MOTIF: cysteine residue (6)
histidine residue
Zn+2-binding site
Breakpoint {470-471}
LIM domain {474-534}
MOTIF: cysteine residue (6)
histidine residue
Zn+2-binding site
LIM domain {535-603}
MOTIF: cysteine residue (6)
histidine residue
Zn+2-binding site
Database Correlations
OMIM 600700
UniProt Q93052
Pfam PF00412
References
- UniProt :accession Q93052
- Atlas of Genetics and Cytogenetics in Oncology and Haematology
http://atlasgeneticsoncology.org/Genes/LPPID72.html