Reactome | ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells)

Schema > Reaction > Entries

ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) Show undefined attributes

authored	[InstanceEdit:5223335] Jassal, Bijay, 2014-01-06
catalystActivity	[CatalystActivity:5223338] ABC-type vitamin B12 transporter activity of ABCD4:LMBRD1 [lysosomal membrane]
catalystActivityReference	[CatalystActivityReference:9730748] ABC-type vitamin B12 transporter activity of ABCD4:LMBRD1 [lysosomal membrane] The lysosomal protein ABCD4 can transport vitamin B₁₂ across liposomal membranes in vitro
category	transition
compartment	[Compartment:70101] cytosol [Compartment:161320] lysosomal lumen [Compartment:17938] lysosomal membrane
created	[InstanceEdit:5223335] Jassal, Bijay, 2014-01-06
crossReference	[DatabaseIdentifier:12667551] RHEA:17873
dbId	5223313
deleted	[Deleted:9730755] Deletion of instance: 3000238
diseaseReaction	[FailedReaction:R-HSA-5683325] Defective ABCD4:LMBRD1 does not transport Cbl from lysosomal lumen to cytosol - Homo sapiens
displayName	ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells)
edited	[InstanceEdit:5223335] Jassal, Bijay, 2014-01-06
eventOf	[Pathway:R-HSA-9758881] Uptake of dietary cobalamins into enterocytes - Homo sapiens
followingEvent	[Reaction:R-HSA-3095901] ABCC1 transports cytosolic RCbl to extracellular region - Homo sapiens
input	[SimpleEntity:R-ALL-113592] ATP [cytosol] [SimpleEntity:R-ALL-29356] H2O [cytosol] [SimpleEntity:R-ALL-9759390] RCbl [lysosomal lumen]
isChimeric	false
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:3928676] Mutations in ABCD4 cause a new inborn error of vitamin B12 metabolism [LiteratureReference:9758824] Translocation of the ABC transporter ABCD4 from the endoplasmic reticulum to lysosomes requires the escort protein LMBD1 [LiteratureReference:9730745] The lysosomal protein ABCD4 can transport vitamin B₁₂ across liposomal membranes in vitro [LiteratureReference:3000283] Identification of a putative lysosomal cobalamin exporter altered in the cblF defect of vitamin B12 metabolism [LiteratureReference:9758708] Defect in vitamin B12 release from lysosomes: newly described inborn error of vitamin B12 metabolism
maxDepth	1
modified	[InstanceEdit:10996769] Weiser, Joel, 2026-03-16
name	ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) an R-cob(III)alamin(out) + ATP + H2O => ADP + an R-cob(III)alamin(in) + H(+) + phosphate
negativePrecedingEvent	[NegativePrecedingEvent:9764883] Different Cell Type: TCN2:RCbl is degraded to release RCbl
orthologousEvent	[Reaction:R-DDI-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Dictyostelium discoideum [Reaction:R-GGA-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Gallus gallus [Reaction:R-XTR-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Xenopus tropicalis [Reaction:R-DRE-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Danio rerio [Reaction:R-SSC-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Sus scrofa [Reaction:R-BTA-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Bos taurus [Reaction:R-CFA-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Canis familiaris [Reaction:R-MMU-5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - Mus musculus
output	[SimpleEntity:R-ALL-29370] ADP [cytosol] [SimpleEntity:R-ALL-29372] Pi [cytosol] [SimpleEntity:R-ALL-9759394] RCbl [cytosol]
precedingEvent	[BlackBoxEvent:R-HSA-3000243] Unknown lysosomal protease degrades CBLIF:RCbl to release Cbl - Homo sapiens
releaseDate	2014-09-30
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:5621563] Rosenblatt, David, 2014-09-01
revised	[InstanceEdit:9730861] D'Eustachio, Peter, 2021-05-11
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-5223313
summation	[Summation:5223327] In the mucosal cells of the distal ileum, ATP binding casset...
updateTrackers	[UpdateTracker:9784867] Update Tracker - [Reaction:5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - v52:[modifyText] [UpdateTracker:9776137] Update Tracker - [Reaction:5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - v77:[addLiteratureReference, modifyText] [UpdateTracker:9775061] Update Tracker - [Reaction:5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - v80:[addLiteratureReference, add_removeInput, add_removeOutput, modifyText]

Referrals

(updatedInstance)	[UpdateTracker:9784867] Update Tracker - [Reaction:5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - v52:[modifyText] [UpdateTracker:9776137] Update Tracker - [Reaction:5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - v77:[addLiteratureReference, modifyText] [UpdateTracker:9775061] Update Tracker - [Reaction:5223313] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) - v80:[addLiteratureReference, add_removeInput, add_removeOutput, modifyText]
(replacementInstances)	[Deleted:9730755] Deletion of instance: 3000238
(normalReaction)	[FailedReaction:R-HSA-5683325] Defective ABCD4:LMBRD1 does not transport Cbl from lysosomal lumen to cytosol
(hasEvent)	[Pathway:R-HSA-9758881] Uptake of dietary cobalamins into enterocytes
(precedingEvent)	[NegativePrecedingEvent:9764873] Different Cell Type: ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol (gut mucosal cells) [Reaction:R-HSA-3095901] ABCC1 transports cytosolic RCbl to extracellular region

Cite Us!