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Rho guanine nucleotide exchange factor 12; leukemia-associated RhoGEF (ARHGEF12, KIAA0382, LARG)

Function: - may play a role in the regulation of RhoA GTPase by guanine nucleotide-binding alpha-12 (GNA12) & guanine nucleotide-binding alpha-13 (GNA13) - acts as guanine nucleotide exchange factor (GEF) for RhoA GTPase & may act as GTPase-activating protein (GAP) for GNA12 & GNA13 - phosphorylated upon DNA damage, probably by ATM or ATR - interacts with GNA12 & GNA13, probably through the RGS- like domain - interacts with RHOA, PLXNB1 & PLXNB2 - interacts through its PDZ domain with IGF1R beta subunit Structure: - contains 1 DH (DBL-homology) domain - contains 1 PDZ domain (DHR domain) - contains 1 PH domain - contains 1 RGSL (RGS-like) domain Compartment: - cytoplasm (probable) - membrane (probable) - translocated to the membrane upon stimulation (probable) Alternative splicing: named isoforms=2 Expression: - ubiquitously expressed - isoform 2 is found in jejunum & testis Pathology: - chromosomal translocation t(11;11)(q23;23) involving ARHGEF12 with MLL may be a cause of acute leukemia

Interactions

molecular events

General

phosphoprotein rho guanine nucleotide exchange factor

Properties

SIZE: entity length = 1544 aa MW = 173 kD COMPARTMENT: cytoplasm MOTIF: Ser phosphorylation site {S22} Ser phosphorylation site {S41} PDZ domain NAME: PDZ domain SITE: 72-151 coiled coil {194-262} breakpoint {308-309} Ser phosphorylation site {S341} RGSL {367-558} Ser phosphorylation site {S637} Thr phosphorylation site {T736} Dbl homology domain {787-977} PH domain {1019-1132} Thr phosphorylation site {T1286} Ser phosphorylation site {S1288} Ser phosphorylation site {S1327}

Database Correlations

OMIM 604763 UniProt Q9NZN5 PFAM correlations Entrez Gene 23365 Kegg hsa:23365

References

  1. UniProt :accession Q9NZN5
  2. Atlas of Genetics & Cytogenetics in Oncology & Haematology http://atlasgeneticsoncology.org/genes/LARGID243.html

Component-of

molecular complex