Table 1.

Characteristics of BCOR and BCORL1 genes and proteins

CharacteristicsBCOR geneBCORL1 gene
Location Chromosome X (band Xp11.4) Chromosome X (band Xp26.1) 
No. of exons 15 13 
Associated genetic syndrome OFCD Shukla-Vernon (SHUVER) 
Mutations Frameshifts, nonsense, and missense* Frameshifts, nonsense, and missense*, 
Translocations Rare (APL)* No 
ITD of PUFD (solid tumors) Yes No 
Characteristics BCOR protein BCORL1 protein 
Length 1755 amino acids 1711 amino acids 
Subcellular location Nucleosol and nuclear dots of various size Speckle-like nuclear dots of consistent size 
Major protein domains BCL6-binding domain, PUFD motif, MLLT3-binding domain
Tandem ankyrin repeats 
CtBP1-binding site, PUFD motif, 2 LXXLL motifs, tandem ankyrin repeats 
PUFD motif Disordered Ordered 
Function Transcriptional corepressor Transcriptional corepressor 
Interactors BCL6, HDACs class I and II, MLLT3, FXBL10/JHDM1B, MLLT1/ENI, ZBTB5, SP1, ZBTB2, ZBTB7A/Pokemon, PCGF1, RING1A/B
KDM2D 
HDACs class I and II, CTBP1, PCGF1 
CharacteristicsBCOR geneBCORL1 gene
Location Chromosome X (band Xp11.4) Chromosome X (band Xp26.1) 
No. of exons 15 13 
Associated genetic syndrome OFCD Shukla-Vernon (SHUVER) 
Mutations Frameshifts, nonsense, and missense* Frameshifts, nonsense, and missense*, 
Translocations Rare (APL)* No 
ITD of PUFD (solid tumors) Yes No 
Characteristics BCOR protein BCORL1 protein 
Length 1755 amino acids 1711 amino acids 
Subcellular location Nucleosol and nuclear dots of various size Speckle-like nuclear dots of consistent size 
Major protein domains BCL6-binding domain, PUFD motif, MLLT3-binding domain
Tandem ankyrin repeats 
CtBP1-binding site, PUFD motif, 2 LXXLL motifs, tandem ankyrin repeats 
PUFD motif Disordered Ordered 
Function Transcriptional corepressor Transcriptional corepressor 
Interactors BCL6, HDACs class I and II, MLLT3, FXBL10/JHDM1B, MLLT1/ENI, ZBTB5, SP1, ZBTB2, ZBTB7A/Pokemon, PCGF1, RING1A/B
KDM2D 
HDACs class I and II, CTBP1, PCGF1 
*

Sometimes may co-occur in AML and MDS.

Rare variant of acute promyelocytic leukemia (APL).

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