Reactome | ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol

Schema > Reaction > Entries

ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol Show undefined attributes

authored	[InstanceEdit:9759205] D'Eustachio, Peter, 2021-11-29
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:9759205] D'Eustachio, Peter, 2021-11-29
crossReference	[DatabaseIdentifier:12667551] RHEA:17873
dbId	9759206
displayName	ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol
edited	[InstanceEdit:9759205] D'Eustachio, Peter, 2021-11-29
eventOf	[Pathway:R-HSA-9758890] Transport of RCbl within the body - Homo sapiens
followingEvent	[Reaction:R-HSA-3149519] MMACHC decyanates CNCbl - Homo sapiens [Reaction:R-HSA-3095889] MMACHC dealkylates RCbl - 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 an R-cob(III)alamin(out) + ATP + H2O => ADP + an R-cob(III)alamin(in) + H(+) + phosphate
negativePrecedingEvent	[NegativePrecedingEvent:9764892] Different Cell Type: Unknown lysosomal protease degrades CBLIF:RCbl to release Cbl
orthologousEvent	[Reaction:R-DDI-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Dictyostelium discoideum [Reaction:R-GGA-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Gallus gallus [Reaction:R-XTR-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Xenopus tropicalis [Reaction:R-DRE-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Danio rerio [Reaction:R-SSC-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Sus scrofa [Reaction:R-BTA-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Bos taurus [Reaction:R-CFA-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - Canis familiaris [Reaction:R-MMU-9759206] ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol - 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-3000263] TCN2:RCbl is degraded to release RCbl - Homo sapiens
releaseDate	2022-03-23
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:9764895] Hill, David P, 2022-02-14
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-9759206
summation	[Summation:9759201] In cells throughout the body that have taken up TCN2:RCbl fr...

Referrals

(hasEvent)	[Pathway:R-HSA-9758890] Transport of RCbl within the body
(precedingEvent)	[Reaction:R-HSA-3149519] MMACHC decyanates CNCbl [Reaction:R-HSA-3095889] MMACHC dealkylates RCbl [NegativePrecedingEvent:9764886] Different Cell Type: ABCD4:LMBRD1 transports RCbl from lysosomal lumen to cytosol