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Schema
>
Reaction
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Entries
methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+
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catalystActivity
[CatalystActivity:70892] methylmalonate-semialdehyde dehydrogenase (acylating, NAD) activity of ALDH6A1 [mitochondrial matrix]
category
transition
compartment
[Compartment:5460] mitochondrial matrix
crossReference
[DatabaseIdentifier:12610559] Rhea:76628
[DatabaseIdentifier:9925935] RHEA:76628
dbId
70893
displayName
methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+
eventOf
[Pathway:R-HSA-70895] Branched-chain amino acid catabolism - Homo sapiens
followingEvent
[Reaction:R-HSA-71031] propionyl-CoA + CO2 + ATP <=> D-methylmalonyl-CoA + ADP + orthophosphate - Homo sapiens
input
[SimpleEntity:R-ALL-30004] 2M3OPROA [mitochondrial matrix]
[SimpleEntity:R-ALL-29374] CoA-SH [mitochondrial matrix]
[SimpleEntity:R-ALL-113521] H2O [mitochondrial matrix]
[SimpleEntity:R-ALL-113526] NAD+ [mitochondrial matrix]
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:508470] Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency
[LiteratureReference:508475] CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution
maxDepth
1
modified
[InstanceEdit:10948619] Weiser, Joel, 2025年08月27日
name
methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+
(S)-2-methyl-3-oxopropanoate + CoA + H2O + NAD(+) => H(+) + hydrogencarbonate + NADH + propanoyl-CoA
negativePrecedingEvent
[NegativePrecedingEvent:9930371] beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA
orthologousEvent
[Reaction:R-DRE-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Danio rerio
[Reaction:R-DDI-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Dictyostelium discoideum
[Reaction:R-CEL-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Caenorhabditis elegans
[Reaction:R-DME-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Drosophila melanogaster
[Reaction:R-GGA-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Gallus gallus
[Reaction:R-XTR-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Xenopus tropicalis
[Reaction:R-SSC-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Sus scrofa
[Reaction:R-BTA-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Bos taurus
[Reaction:R-CFA-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Canis familiaris
[Reaction:R-RNO-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Rattus norvegicus
[Reaction:R-MMU-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Mus musculus
output
[SimpleEntity:R-ALL-113529] H+ [mitochondrial matrix]
[SimpleEntity:R-ALL-29896] HCO3- [mitochondrial matrix]
[SimpleEntity:R-ALL-29362] NADH [mitochondrial matrix]
[SimpleEntity:R-ALL-70843] PROP-CoA [mitochondrial matrix]
precedingEvent
[Reaction:R-HSA-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Homo sapiens
previousReviewStatus
[ReviewStatus:9821385] four stars
releaseDate
2004年07月06日
reviewStatus
[ReviewStatus:9821382] five stars
reviewed
[InstanceEdit:9917333] Hill, David P, 2024年08月02日
[InstanceEdit:9927657] D'Eustachio, Peter, 2024年11月08日
schemaClass
Reaction
species
[Species:48887] Homo sapiens
stId
R-HSA-70893
summation
[Summation:178951] Mitochondrial methylmalonate semialdehyde dehydrogenase (ALD...
updateTrackers
[UpdateTracker:9790928] Update Tracker - [Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ - v32:[add_removeLiteratureReference, modifyText]
[UpdateTracker:9919402] Update Tracker - [Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ - v90:[removeInput]
[UpdateTracker:9928397] Update Tracker - [Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - v91:[add_removeOutput]
Referrals
(updatedInstance)
[UpdateTracker:9790928] Update Tracker - [Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ - v32:[add_removeLiteratureReference, modifyText]
[UpdateTracker:9919402] Update Tracker - [Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA => propionyl-CoA + CO2 + NADH + H+ - v90:[removeInput]
[UpdateTracker:9928397] Update Tracker - [Reaction:70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - v91:[add_removeOutput]
(hasEvent)
[Pathway:R-HSA-70895] Branched-chain amino acid catabolism
(precedingEvent)
[NegativePrecedingEvent:9930391] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+
[Reaction:R-HSA-71031] propionyl-CoA + CO2 + ATP <=> D-methylmalonyl-CoA + ADP + orthophosphate
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