Reactome | beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+

Schema > Reaction > Entries

beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ Show undefined attributes

catalystActivity	[CatalystActivity:70884] 3-hydroxyisobutyrate dehydrogenase activity of HIBADH tetramer [mitochondrial matrix]
category	transition
compartment	[Compartment:5460] mitochondrial matrix
crossReference	[DatabaseIdentifier:12667554] RHEA:31276 [DatabaseIdentifier:9925941] RHEA:31276
dbId	70885
displayName	beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+
eventOf	[Pathway:R-HSA-70895] Branched-chain amino acid catabolism - Homo sapiens
followingEvent	[Reaction:R-HSA-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Homo sapiens
input	[SimpleEntity:R-ALL-573404] bHIBA [mitochondrial matrix] [SimpleEntity:R-ALL-113526] NAD+ [mitochondrial matrix]
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:70886] Purification and properties of beta-hydroxyisobutyric dehydrogenase
maxDepth	1
modified	[InstanceEdit:10996769] Weiser, Joel, 2026-03-16
name	beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ (S)-3-hydroxy-2-methylpropanoate + NAD(+) => (S)-2-methyl-3-oxopropanoate + H(+) + NADH
negativePrecedingEvent	[NegativePrecedingEvent:9908870] Futile Cycle: methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+
orthologousEvent	[Reaction:R-DDI-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Dictyostelium discoideum [Reaction:R-CEL-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Caenorhabditis elegans [Reaction:R-DME-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Drosophila melanogaster [Reaction:R-GGA-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Gallus gallus [Reaction:R-XTR-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Xenopus tropicalis [Reaction:R-DRE-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Danio rerio [Reaction:R-SSC-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Sus scrofa [Reaction:R-BTA-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Bos taurus [Reaction:R-CFA-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Canis familiaris [Reaction:R-RNO-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Rattus norvegicus [Reaction:R-MMU-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Mus musculus
output	[SimpleEntity:R-ALL-30004] 2M3OPROA [mitochondrial matrix] [SimpleEntity:R-ALL-113529] H+ [mitochondrial matrix] [SimpleEntity:R-ALL-29362] NADH [mitochondrial matrix]
precedingEvent	[Reaction:R-HSA-70881] beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA - Homo sapiens
releaseDate	2004-07-06
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:532425] Jassal, Bijay, 2010-02-26
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-70885
summation	[Summation:178504] Mitochondrial 3-hydroxyisobutyrate dehydrogenase (HIBADH) ca...
updateTrackers	[UpdateTracker:9791168] Update Tracker - [Reaction:70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - v32:[modifyText, removeLiteratureReference] [UpdateTracker:9790550] Update Tracker - [Reaction:70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - v33:[add_removeInput]

Referrals

(updatedInstance)	[UpdateTracker:9791168] Update Tracker - [Reaction:70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - v32:[modifyText, removeLiteratureReference] [UpdateTracker:9790550] Update Tracker - [Reaction:70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - v33:[add_removeInput]
(hasEvent)	[Pathway:R-HSA-70895] Branched-chain amino acid catabolism
(precedingEvent)	[Reaction:R-HSA-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ [NegativePrecedingEvent:9908865] Futile Cycle: beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+
(reverseReaction)	[Reaction:R-HSA-508473] methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+