LMPD Database

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LMP006518

UniProt Annotations

Entry Information
Gene NameRab geranylgeranyl transferase, a subunit
Protein EntryPGTA_MOUSE
UniProt IDQ9JHK4
SpeciesMouse
Comments
Comment typeDescription
Alternative ProductsEvent=Alternative splicing; Named isoforms=2; Name=1; IsoId=Q9JHK4-1; Sequence=Displayed; Name=2; IsoId=Q9JHK4-2; Sequence=VSP_009113, VSP_009114;
Catalytic ActivityGeranylgeranyl diphosphate + protein-cysteine = S-geranylgeranyl-protein + diphosphate.
DiseaseNote=Defects in Rabggta are the cause of the gunmetal (gm) phenotype. Mice homozygous for gm have prolonged bleeding, thrombocytopenia and reduced platelet alpha- and delta-granule contents. {ECO:0000269|PubMed:10737774}.
Enzyme RegulationThe enzymatic reaction requires the aid of a Rab escort protein (also called component A), such as CHM. {ECO:0000250}.
FunctionCatalyzes the transfer of a geranylgeranyl moiety from geranylgeranyl diphosphate to both cysteines of Rab proteins with the C-terminal sequence -XXCC, -XCXC and -CCXX, such as RAB1A, RAB3A, RAB5A and RAB7A. {ECO:0000269|PubMed:10737774}.
SimilarityBelongs to the protein prenyltransferase subunit alpha family. {ECO:0000305}.
SimilarityContains 5 LRR (leucine-rich) repeats. {ECO:0000305}.
SimilarityContains 6 PFTA repeats. {ECO:0000255|PROSITE- ProRule:PRU00488}.
SubunitHeterotrimer composed of RABGGTA, RABGGTB and CHM; within this trimer, RABGGTA and RABGGTB form the catalytic component B, while CHM (component A) mediates peptide substrate binding. The Rab GGTase dimer (RGGT) interacts with CHM (component A) prior to Rab protein binding; the association is stabilized by geranylgeranyl pyrophosphate (GGpp). The CHM:RGGT:Rab complex is destabilized by GGpp (By similarity). {ECO:0000250}.