LPP (lipoma preferred partner)

HGNC (Hugo)	LPP
HGNC Alias name	lipoma preferred partner gene
HGNC Previous name	LIM domain-containing preferred translocation partner in lipoma
LocusID (NCBI)	4026
Atlas_Id	72
Location	3q28  [Link to chromosome band 3q28]
Location_base_pair	Starts at 188212896 and ends at 188890671 bp from pter ( according to hg19-Feb_2009)  [Mapping LPP.png]
	Probe(s) - Courtesy Mariano Rocchi, Resources for Molecular Cytogenetics

Description	At least 11 exons; predicted start codon in exon 3, stop codon in exon 11; the protein coding region is covered by the overlapping "CEPH Mark 1" YAC clones 135H6 and 192B10 (start codon in 135H6, stop codon in 192B10) and is dispersed over at least 400 kb genomic DNA; the LIM domains are encoded by separate exons: LIM 1 is encoded by exon 8, LIM 2 by exon 9, and LIM 3 by exon 10 and part of exon 11.
Transcription	mRNA: ubiquitously: > 10 kb; testis: additional transcripts of 1.8 kb and 1.25 kb
Pseudogene	no pseudogenes

Note
Entity	solitary lipomas
Disease	Benign tumors of adipose tissue.
Prognosis	Can be surgically removed with no recurrence in most cases.
Cytogenetics	More than 60% of solitary lipomas have an aberrant karyotype; 2/3 of these carry 12q15 rearrangements, most often translocations, affecting the HMGA2 gene; 1/4 of the latter have chromosomal region 3q27-q28 (containing LPP) as 12q15 translocation partner as such creating an HMGA2/LPP fusion gene.
Hybrid/Mutated Gene	HMGA2/LPP hybrid gene containing the first three exons of HMGA2 and exons 8-11 or 9-11 of LPP; under the regulation of the HMGA2 promoter.
Abnormal Protein	HMGA2/LPP fusion transcripts encode the three DNA-binding domains of HMGA2 followed by two LIM domains (LIM 2 and LIM 3) or a portion of the proline-rich region and all three LIM domains of LPP.
Entity	Pulmonary chondroid hamartomas
Disease	Benign mesenchymal tumors of the lung.
Prognosis	good
Cytogenetics	More than 70% of pulmonary chondroid hamartomas have an aberrant karyotype; 70% of these carry 12q15 rearrangements, most often translocations, affecting the HMGA2 gene; 1/8 of the latter have chromosomal region 3q27-q28 (containing LPP) as 12q15 translocation partner as such creating an HMGA2/LPP fusion gene.
Hybrid/Mutated Gene	HMGA2/LPP hybrid gene containing the first three exons of HMGA2 and exons 9-11 of LPP; under the regulation of the HMGA2 promoter.
Abnormal Protein	HMGA2/LPP fusion transcripts encode the three DNA-binding domains of HMGA2 followed by the two most carboxy-terminal LIM domains (LIM 2 and LIM 3) of LPP.
Entity	Parosteal lipoma
Disease	Rare deep-seated benign tumor of adipose tissue comprising less than 0.5% of all lipomas; parosteal lipomas exhibit a contiguous relationship with the periostium; because of their intimate relationship to the bone, they are considered as lipomas of bone.
Prognosis	Most often asymptomatic; in some cases: loss of motor and/or sensory function as a result of the compression or stretching of a nerve.
Cytogenetics	One case reported with rearrangement of LPP t(3;12)(q28;q14).
Hybrid/Mutated Gene	HMGA2/LPP hybrid gene containing the first three exons of HMGA2 and exons 9-11 of LPP; under the regulation of the HMGA2 promoter.
Abnormal Protein	HMGA2/LPP fusion transcripts encode the three DNA-binding domains of HMGA2 followed by the two most carboxy-terminal LIM domains (LIM 2 and LIM 3) of LPP.
Entity	Soft tissue chondroma
Disease	Benign tumor of cartilage; rare entity.
Cytogenetics	Only 31 cases with abnormal karyotypes have been reported (11-2003); 12q15 nonrandomly involved; one case reported with rearrangement of LPP t(3;12)(q27;q15).
Hybrid/Mutated Gene	HMGA2/LPP hybrid gene containing the first three exons of HMGA2 and exons 9-11 of LPP; under the regulation of the HMGA2 promoter.
Abnormal Protein	HMGA2/LPP fusion transcripts encode the three DNA-binding domains of HMGA2 followed by the two most carboxy-terminal LIM domains (LIM 2 and LIM 3) of LPP.
Entity	AML-M5
Disease	Secondary leukemia following treatment with DNA topoisomerase II inhibitors.
Cytogenetics	MLL gene on 11q23 frequently involved; one case reported with rearrangement of LPP t(3;11)(q28;q23).
Hybrid/Mutated Gene	MLL/LPP hybrid gene containing the first 8 exons of MLL and exons 9-11 of LPP; under the regulation of the MLL promoter.
Abnormal Protein	MLL/LPP fusion transcripts encode the three DNA-binding domains and the methyltransferase-like domain of MLL followed by the two most carboxy-terminal LIM domains (LIM 2 and LIM 3) of LPP.

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