Reactome | UniProt:P51159 RAB27A

chain	initiator methionine:1 chain:2-221
checksum	4A6A0C8C5CC41A20
comment	FUNCTION The small GTPases Rab are key regulators of intracellular membrane trafficking, from the formation of transport vesicles to their fusion with membranes. Rabs cycle between an inactive GDP-bound form and an active GTP-bound form that is able to recruit to membranes different sets of downstream effectors directly responsible for vesicle formation, movement, tethering and fusion (PubMed:30771381). RAB27A regulates homeostasis of late endocytic pathway, including endosomal positioning, maturation and secretion (PubMed:30771381). Plays a role in cytotoxic granule exocytosis in lymphocytes. Required for both granule maturation and granule docking and priming at the immunologic synapse (PubMed:18812475).CATALYTIC ACTIVITY GTP + H2O = GDP + phosphate + H(+)COFACTOR Regulated by guanine nucleotide exchange factors (GEFs) including DENND10, which promote the exchange of bound GDP for free GTP (Probable). Regulated by GTPase activating proteins (GAPs) which increase the GTP hydrolysis activity. Inhibited by GDP dissociation inhibitors (GDIs) (Probable).SUBUNIT Binds SYTL1, SLAC2B, MYRIP, SYTL3, SYTL4 and SYTL5. Interacts with RPH3A and RPH3A (By similarity). Interacts with MLPH (PubMed:12062444). Interacts with SYTL2 (PubMed:18812475, PubMed:35308027). Interacts with UNC13D (PubMed:15548590, PubMed:17237785). Does not interact with the BLOC-3 complex (heterodimer of HPS1 and HPS4) (PubMed:20048159). Interacts (GDP-bound form preferentially) with DENND10 (PubMed:30771381). Interacts with FBXO28; this interaction promotes RAB27A ubiquitination and proteasomal degradation (By similarity).INTERACTION Identified by mass spectrometry in melanosome fractions from stage I to stage IV (PubMed:12643545, PubMed:17081065). Localizes to endosomal exocytic vesicles (PubMed:17237785).ALTERNATIVE PRODUCTS Found in all the examined tissues except in brain. Low expression was found in thymus, kidney, muscle and placenta. Detected in melanocytes, and in most tumor cell lines examined. Expressed in cytotoxic T-lymphocytes (CTL) and mast cells.DOMAIN Switch I, switch II and the interswitch regions are characteristic of Rab GTPases and mediate the interactions with Rab downstream effectors. The switch regions undergo conformational changes upon nucleotide binding which drive interaction with specific sets of effector proteins, with most effectors only binding to GTP-bound Rab.PTM Ubiquitinated in a FBXO28-dependent manner; leading to proteasomal degradation.DISEASE The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the small GTPase superfamily. Rab family.ONLINE INFORMATION RAB27A mutation db
created	[InstanceEdit:400710] Schmidt, EE, 2009-03-25 05:33:35
crossReference	[DatabaseIdentifier:11489800] ENSEMBL:ENSG00000069974 [DatabaseIdentifier:12407774] RefSeq:NP_001425901.1 [DatabaseIdentifier:12407784] RefSeq:NP_004571.2 [DatabaseIdentifier:12407775] RefSeq:NP_001425904.1 [DatabaseIdentifier:12407770] RefSeq:XP_047288879.1 [DatabaseIdentifier:12407779] RefSeq:NP_001425908.1 [DatabaseIdentifier:12407780] RefSeq:NP_001425907.1 [DatabaseIdentifier:12407769] RefSeq:XP_054234531.1 [DatabaseIdentifier:12407773] RefSeq:NP_001425902.1 [DatabaseIdentifier:12407772] RefSeq:NP_001425910.1 [DatabaseIdentifier:12407783] RefSeq:NP_899058.1 [DatabaseIdentifier:12407786] RefSeq:NP_001425899.1 [DatabaseIdentifier:12407781] RefSeq:NP_899059.1 [DatabaseIdentifier:12407777] RefSeq:NP_001425906.1 [DatabaseIdentifier:12407771] RefSeq:XP_047288876.1 [DatabaseIdentifier:12407768] RefSeq:XP_054234535.1 [DatabaseIdentifier:12407785] RefSeq:NP_899057.1 [DatabaseIdentifier:12407767] RefSeq:XP_047288874.1 [DatabaseIdentifier:12407778] RefSeq:NP_001425905.1 [DatabaseIdentifier:12407776] RefSeq:NP_001425903.1 [DatabaseIdentifier:12407782] RefSeq:NP_001425909.1 [DatabaseIdentifier:12283879] Pharos - Targets:P51159 [DatabaseIdentifier:12242840] PRO:P51159 [DatabaseIdentifier:12168008] PDB:6HUF [DatabaseIdentifier:12147898] PDB:7OPP [DatabaseIdentifier:12147900] PDB:8P3I [DatabaseIdentifier:12147903] PDB:8P3G [DatabaseIdentifier:12147904] PDB:8P3H [DatabaseIdentifier:12147899] PDB:7OPQ [DatabaseIdentifier:12147905] PDB:8P3K [DatabaseIdentifier:12147902] PDB:8P3J [DatabaseIdentifier:12147897] PDB:7OPR [DatabaseIdentifier:12138077] Orphanet:RAB27A [DatabaseIdentifier:12123560] OpenTargets:ENSG00000069974 [DatabaseIdentifier:11595954] ENSEMBL:ENSP00000455012 [DatabaseIdentifier:11595952] ENSEMBL:ENSP00000379601 [DatabaseIdentifier:11595953] ENSEMBL:ENSP00000337761 [DatabaseIdentifier:11595956] ENSEMBL:ENSP00000513369 [DatabaseIdentifier:11595955] ENSEMBL:ENSP00000513368 [DatabaseIdentifier:11595957] ENSEMBL:ENSP00000456059 [DatabaseIdentifier:11718654] ENSEMBL:ENST00000569493 [DatabaseIdentifier:11718653] ENSEMBL:ENST00000336787 [DatabaseIdentifier:11718651] ENSEMBL:ENST00000697642 [DatabaseIdentifier:11718652] ENSEMBL:ENST00000697643 [DatabaseIdentifier:11718656] ENSEMBL:ENST00000564609 [DatabaseIdentifier:11718655] ENSEMBL:ENST00000396307 [DatabaseIdentifier:11781245] Guide to Pharmacology - Targets:2916 [DatabaseIdentifier:11785962] GeneCards:RAB27A [DatabaseIdentifier:11801469] GlyGen:P51159 [DatabaseIdentifier:11839046] HPA:ENSG00000069974-RAB27A
databaseName	UniProt
dbId	404270
description	recommendedName: Ras-related protein Rab-27A shortName: Rab-27 ecNumber evidence="19 20"3.6.5.2 alternativeName: GTP-binding protein Ram
displayName	UniProt:P51159 RAB27A
geneName	RAB27A RAB27
identifier	P51159
isSequenceChanged	false
keyword	3D-structure Acetylation Alternative splicing Direct protein sequencing Disease variant Disulfide bond Endosome Exocytosis GTP-binding Hydrolase Lipoprotein Lysosome Membrane Methylation Nucleotide-binding Phosphoprotein Prenylation Proteomics identification Reference proteome Ubl conjugation
modified	[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
moleculeType	Protein
name	RAB27A
otherIdentifier	11725424_a_at 11725425_s_at 11725426_a_at 11742071_x_at 11753165_a_at 11758934_x_at 16809596 209514_PM_s_at 209514_s_at 209515_PM_s_at 209515_s_at 210951_PM_x_at 210951_x_at 222294_PM_s_at 222294_s_at 235766_PM_x_at 235766_x_at 3625272 3625273 3625274 3625275 3625276 3625277 3625278 3625279 3625282 3625283 3625284 3625286 3625288 3625292 3625294 3625295 3625296 3625297 3625301 3625302 3625303 3625312 3625316 3625321 40669_at 55539_at 5873 65080_r_at 68324_at 7989023 809_at A_23_P54373 A_24_P373174 GE59251 GE851846 GO:0000166 GO:0001750 GO:0002376 GO:0003924 GO:0003925 GO:0005515 GO:0005525 GO:0005576 GO:0005764 GO:0005768 GO:0005770 GO:0005794 GO:0005829 GO:0006887 GO:0007596 GO:0009306 GO:0010628 GO:0016020 GO:0016192 GO:0016324 GO:0016787 GO:0019003 GO:0019882 GO:0019904 GO:0030141 GO:0030154 GO:0030318 GO:0030425 GO:0031410 GO:0031489 GO:0032400 GO:0032402 GO:0032585 GO:0033093 GO:0033162 GO:0035580 GO:0036257 GO:0042470 GO:0043316 GO:0043320 GO:0043473 GO:0045921 GO:0048489 GO:0050766 GO:0051875 GO:0051904 GO:0070062 GO:0070382 GO:0071985 GO:0097278 GO:0098772 GO:1903307 GO:1903428 GO:1903435 GO:1990182 HMNXSV003014389 HMNXSV003016773 Hs.165337.0.A1_3p_a_at Hs.165337.1.A1_3p_at Hs.165337.1.A1_3p_x_at Hs.50477.1.S1_3p_a_at ILMN_1665859 ILMN_1681461 ILMN_1699878 ILMN_2329773 PH_hs_0023582 TC15001465.hg U57094_at g4887230_3p_a_at
physicalEntity	[EntityWithAccessionedSequence:R-HSA-6799609] RAB27A [specific granule lumen] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-6799682] RAB27A [extracellular region] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-8877281] GGC-RAB27A [melanosome membrane] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-3008674] RAB27A [cytosol] - Homo sapiens [EntityWithAccessionedSequence:R-HSA-8870427] GGC-RAB27A [cytosol] - Homo sapiens
referenceDatabase	[ReferenceDatabase:2] UniProt
referenceGene	[ReferenceDNASequence:12068926] NCBI Gene:5873 RAB27A [ReferenceDNASequence:12697620] UCSC:P51159 RAB27A [ReferenceDNASequence:12109065] OMIM:603868 RAB27A [ReferenceDNASequence:ensembl:ENSG00000069974] ENSEMBL:ENSG00000069974 RAB27A [ReferenceDNASequence:11355044] BioGPS Gene:5873 RAB27A [ReferenceDNASequence:11394012] COSMIC - genes:RAB27A RAB27A [ReferenceDNASequence:11410684] CTD Gene:5873 RAB27A [ReferenceDNASequence:11430056] dbSNP Gene:5873 RAB27A [ReferenceDNASequence:11817593] HGNC:9766 RAB27A [ReferenceDNASequence:11894552] KEGG:hsa:5873 RAB27A [ReferenceDNASequence:11984833] Monarch:5873 RAB27A
referenceTranscript	[ReferenceRNASequence:12589458] RefSeq:NM_001438974.1 RAB27A [ReferenceRNASequence:12589472] RefSeq:XM_054378560.1 RAB27A [ReferenceRNASequence:12589475] RefSeq:XM_054378556.1 RAB27A [ReferenceRNASequence:12589469] RefSeq:NM_183235.3 RAB27A [ReferenceRNASequence:12589457] RefSeq:NM_001438973.1 RAB27A [ReferenceRNASequence:12589464] RefSeq:NM_001438978.1 RAB27A [ReferenceRNASequence:12589459] RefSeq:NM_001438970.1 RAB27A [ReferenceRNASequence:12589476] RefSeq:NM_001438972.1 RAB27A [ReferenceRNASequence:12589468] RefSeq:NM_183234.3 RAB27A [ReferenceRNASequence:12589462] RefSeq:XM_047432918.1 RAB27A [ReferenceRNASequence:12589471] RefSeq:NM_004580.4 RAB27A [ReferenceRNASequence:12589460] RefSeq:NM_001438975.1 RAB27A [ReferenceRNASequence:12589463] RefSeq:NM_001438977.1 RAB27A [ReferenceRNASequence:12589473] RefSeq:NM_001438980.1 RAB27A [ReferenceRNASequence:12589466] RefSeq:XM_047432923.1 RAB27A [ReferenceRNASequence:12589465] RefSeq:NM_001438979.1 RAB27A [ReferenceRNASequence:12589461] RefSeq:NM_001438976.1 RAB27A [ReferenceRNASequence:12589474] RefSeq:NM_001438981.1 RAB27A [ReferenceRNASequence:12589470] RefSeq:NM_183236.3 RAB27A [ReferenceRNASequence:12589467] RefSeq:XM_047432920.1 RAB27A
schemaClass	ReferenceGeneProduct
secondaryIdentifier	RB27A_HUMAN O00195 Q6FI40 Q9UIR9 Q9Y5U3
sequenceLength	221
species	[Species:48887] Homo sapiens
stId	uniprot:P51159
url	http://purl.uniprot.org/uniprot/P51159

(referenceEntity)	[EntityWithAccessionedSequence:R-HSA-6799609] RAB27A [specific granule lumen] [EntityWithAccessionedSequence:R-HSA-6799682] RAB27A [extracellular region] [EntityWithAccessionedSequence:R-HSA-8877281] GGC-RAB27A [melanosome membrane] [EntityWithAccessionedSequence:R-HSA-3008674] RAB27A [cytosol] [EntityWithAccessionedSequence:R-HSA-8870427] GGC-RAB27A [cytosol]
(referenceSequence)	[ModifiedResidue:8870404] S-geranylgeranyl-L-cysteine at 219 [ModifiedResidue:8870408] S-geranylgeranyl-L-cysteine at 221
(interactor)	[UndirectedInteraction:11170287] UniProt:Q92696 <-> UniProt:P51159 (IntAct) [UndirectedInteraction:11202487] UniProt:P51159 <-> UniProt:Q9BV36 (IntAct) [UndirectedInteraction:11202489] UniProt:P51159 <-> UniProt:Q96C24 (IntAct) [UndirectedInteraction:11202493] UniProt:P51159 <-> UniProt:Q4VX76 (IntAct) [UndirectedInteraction:11202495] UniProt:P51159 <-> UniProt:Q8IYJ3 (IntAct) [UndirectedInteraction:11202497] UniProt:P51159 <-> UniProt:Q70J99 (IntAct) [UndirectedInteraction:11202499] UniProt:P51159 <-> UniProt:Q8TDW5-2 (IntAct) [UndirectedInteraction:11202501] UniProt:P51159 <-> UniProt:Q8NFW9 (IntAct) [UndirectedInteraction:11202503] UniProt:P51159 <-> UniProt:P24386 (IntAct) [UndirectedInteraction:11202507] UniProt:P51159 <-> UniProt:Q8TDW5 (IntAct) [UndirectedInteraction:11202509] UniProt:P51159 <-> UniProt:P26374 (IntAct) [UndirectedInteraction:11202511] UniProt:P51159 <-> UniProt:Q9HCH5 (IntAct) [UndirectedInteraction:11202513] UniProt:P51159 <-> UniProt:P04062 (IntAct) [UndirectedInteraction:11202515] UniProt:P51159 <-> UniProt:O00194 (IntAct)

UniProt:P51159 RAB27A Show undefined attributes

Referrals