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Details on Person FKRP dimer transfers RboP to matriglycan chain

Class:IdReaction:9940756
_displayNameFKRP dimer transfers RboP to matriglycan chain
_doReleaseTRUE
_timestamp2025-05-05 07:09:46
authored[InstanceEdit:9940797] Stephan, Ralf, 2025-03-02
catalystActivity[CatalystActivity:9940775] phosphotransferase activity, for other substituted phosphate groups of FKRP:FKTN:RXYLT1 [Golgi membrane]
compartment[Compartment:17963] Golgi lumen
created[InstanceEdit:9940703] Stephan, Ralf, 2025-03-06
crossReference[DatabaseIdentifier:9940667] RHEA:39868
edited[InstanceEdit:9947109] Stephan, Ralf, 2025-05-01
input[SimpleEntity:9940856] CDP-ribitol [Golgi lumen]
[EntityWithAccessionedSequence:9940763] RboP-GalNAc-GlcNAc-Man6P-DAG1 [Golgi lumen] [Homo sapiens]
internalReviewed[InstanceEdit:9944933] D'Eustachio, Peter, 2025-04-08
isChimericFALSE
literatureReference[LiteratureReference:9940675] Cell endogenous activities of fukutin and FKRP coexist with the ribitol xylosyltransferase, TMEM5
[LiteratureReference:9940676] Identification of a Post-translational Modification with Ribitol-Phosphate and Its Defect in Muscular Dystrophy
[LiteratureReference:9940768] Crystal structures of fukutin-related protein (FKRP), a ribitol-phosphate transferase related to muscular dystrophy
[LiteratureReference:9940682] Mutations in the FKRP gene can cause muscle-eye-brain disease and Walker-Warburg syndrome
[LiteratureReference:9940806] Mutations in the fukutin-related protein gene (FKRP) cause a form of congenital muscular dystrophy with secondary laminin alpha2 deficiency and abnormal glycosylation of alpha-dystroglycan
[LiteratureReference:9940665] Mutations in the fukutin-related protein gene (FKRP) identify limb girdle muscular dystrophy 2I as a milder allelic variant of congenital muscular dystrophy MDC1C
modified[InstanceEdit:9944934] Stephan, Ralf, 2025-04-08
[InstanceEdit:9947108] Stephan, Ralf, 2025-05-01
[InstanceEdit:9947328] Stephan, Ralf, 2025-05-05
nameFKRP dimer transfers RboP to matriglycan chain
output[SimpleEntity:727810] CMP [Golgi lumen]
[SimpleEntity:425969] H+ [Golgi lumen]
[EntityWithAccessionedSequence:9940668] RboP-RboP-GalNAc-GlcNAc-Man6P-DAG1 [Golgi lumen] [Homo sapiens]
precedingEvent
previousReviewStatus[ReviewStatus:9821383] three stars
releaseDate2025-06-18
reviewed[InstanceEdit:9947107] Zaru, Rossana, 2025-05-01
reviewStatus[ReviewStatus:9821382] five stars
species[Species:48887] Homo sapiens
stableIdentifier[StableIdentifier:9940888] R-HSA-9940756.1
summation[Summation:9940878] The ribitol-5-phosphate transferase FKRP:Mg2+ dimer subunit ...
(hasEvent)[Pathway:9939291] Matriglycan biosynthesis on DAG1 [Homo sapiens]
(precedingEvent)[Reaction:9940805] RXYLT1 transfers initial Xyl to matriglycan chain [Homo sapiens]
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No pathways have been reviewed or authored by FKRP dimer transfers RboP to matriglycan chain (9940756)
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