Selected-GenAtlas references	SOURCE	GeneCards	NCBI Gene	Swiss-Prot	Orphanet	Ensembl
	HGNC	UniGene	Nucleotide	OMIM		UCSC

Home Page

FLASH GENE

Symbol

SPIB

contributors: mct - updated : 19-11-2010

HGNC name	Spi-B transcription factor (Spi-1/PU.1 related)
HGNC id	11242

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Location	19q13.33      Physical location : 50.922.194 - 50.932.249
Synonym name	transcription factor Spi-B
Synonym symbol(s)	SPI-B

DNA

TYPE	functioning gene
STRUCTURE	10.06 kb     6 Exon(s)

10 Kb 5' upstream gene genomic sequence study

MAPPING

cloned

Y

linked

N

status

provisional

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_003121 6 splicing 1448 28.8 262 nuclear 1996 8670269 variant 2 - splicing 1444 isoform 2 - 177 cytoplasmic 1996 8670269 also called DeltaSpi-B

EXPRESSION

Type	restricted

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol blood / hematopoietic spleen         Cardiovascular heart         Digestive intestine small intestine       Lymphoid/Immune lymph node           tonsils

tissue

System Tissue Tissue level 1 Tissue level 2 Level Pubmed Species Stage Rna symbol Blood / Hematopoietic         Lymphoid

cell lineage	various hematopoietic cell lineage
cell lines
fluid/secretion

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	a conserved ETS domain mediating specific DNA binding
	a weakly acidic N-terminal transactivation domain (TAD)
	a second TAD rich in proline, serine and threonine

HOMOLOGY

interspecies	ortholog to murine Spib
	homolog to C elegans C18E3.9

intraspecies

homolog to Spi-1/PU.1 putative oncogene

Homologene

FAMILY

SPI-group ETS-protein transcription factors family

CATEGORY

transcription factor

SUBCELLULAR LOCALIZATION	intracellular
	intracellular,cytoplasm,cytoskeleton,microtubule
	intracellular,nucleus

basic FUNCTION	regulating transcription from Pol II promoter
	affecting SPIB function by recruiting factors involved in SPIB activity
	key regulator of plasmacytoid dendritic cells development, whereas T-cell development is critically dependent on GATA3
	implicated in signaling pathways downstream of the B cell receptor and is essential for germinal center formation and maintenance
	playing a role in maintenance of germinal center and memory B cells by direct repression of major plasma cell factors and thereby plasma cell differentiation
	may be contributes to plasmacytoid dendritic cells development by promoting TCF4 activity
	important mediator of both B-cell receptor signaling and early T-cell lineage decisions
	also induces the development of plasmacytoid dendritic cells and can mediate IL7R, REL and IFNG–induced CD40 expression
	as specific transcription factors SPI1, and SPIC, regulate follicular B cell differentiation
	SPI1 and SPIB are essential transcriptional regulators of B-cell differentiation as well as novel tumor suppressors in the B-cell lineage

CELLULAR PROCESS

nucleotide, transcription, regulation

PHYSIOLOGICAL PROCESS

text	transcription activator

PATHWAY

metabolism

signaling

a component

INTERACTION

DNA	binding to a purine-rich sequence the PU box (5'-GAGGAA-3') acting as a
	lymphoid-specific enhancer

RNA

small molecule

protein	interacting with IRF4 and with CREBBP, EP300, SPI1-related, JUN and TBP
	direct target gene critically regulated by POU2AF1

cell & other

REGULATION

ASSOCIATED DISORDERS

corresponding disease(s)

Susceptibility

to primary biliary cirrhosis

Variant & Polymorphism SNP	rs3745516 within SPIB strongly associated with primary biliary cirrhosis

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS

mice deficient in both Spib and Spi1 in the B-cell lineage had reduced frequencies of B cells as well as impaired B-cell differentiation, and developed pre-B cell acute lymphoblastic leukemia before 30 weeks of age