Reactome | bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3

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bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 Show undefined attributes

authored	[InstanceEdit:1605804] Jassal, Bijay, 2011-09-21
catalystActivity	[CatalystActivity:1605773] beta-N-acetylhexosaminidase activity of bHEXA [lysosomal lumen]
category	transition
compartment	[Compartment:161320] lysosomal lumen
created	[InstanceEdit:1605804] Jassal, Bijay, 2011-09-21
dbId	1605595
displayName	bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3
edited	[InstanceEdit:1605804] Jassal, Bijay, 2011-09-21
eventOf	[Pathway:R-HSA-9840310] Glycosphingolipid catabolism - Homo sapiens
followingEvent	[Reaction:R-HSA-1605724] NEU1,4 hydrolyze PSAP(195-273):GM3:PE - Homo sapiens [Reaction:R-HSA-1605717] GM2A binds and mobilizes ligands - Homo sapiens
input	[Complex:R-HSA-1605600] GM2A:GM2 [lysosomal lumen] [SimpleEntity:R-ALL-1605715] H2O [lysosomal lumen]
isChimeric	false
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:1605770] Oligosaccharide structure and amino acid sequence of the major glycopeptides of mature human beta-hexosaminidase [LiteratureReference:1605668] Molecular basis of an adult form of beta-hexosaminidase B deficiency with motor neuron disease [LiteratureReference:1605612] A point mutation in the coding sequence of the beta-hexosaminidase alpha gene results in defective processing of the enzyme protein in an unusual GM2-gangliosidosis variant [LiteratureReference:1605652] Purification and characterization of an activator protein for the degradation of glycolipids GM2 and GA2 by hexosaminidase A [LiteratureReference:1660677] Crystallographic structure of human beta-hexosaminidase A: interpretation of Tay-Sachs mutations and loss of GM2 ganglioside hydrolysis [LiteratureReference:9840723] Formation of a ternary complex between GM2 activator protein, GM2 ganglioside and hexosaminidase A [LiteratureReference:9840725] The enzyme-binding region of human GM2-activator protein [LiteratureReference:9840716] Study of interaction of GM2 activator protein with GM2 using circular dichroism and fluorescence spectroscopy [LiteratureReference:9840720] Evidence for two different active sites on human beta-hexosaminidase A. Interaction of GM2 activator protein with beta-hexosaminidase A [LiteratureReference:1605738] A mutation in the gene of a glycolipid-binding protein (GM2 activator) that causes GM2-gangliosidosis variant AB [LiteratureReference:9840312] Emerging concepts of ganglioside metabolism
maxDepth	1
modified	[InstanceEdit:10914110] Weiser, Joel, 2026-03-16
name	bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3
orthologousEvent	[Reaction:R-CEL-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Caenorhabditis elegans [Reaction:R-GGA-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Gallus gallus [Reaction:R-SSC-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Sus scrofa [Reaction:R-BTA-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Bos taurus [Reaction:R-CFA-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Canis familiaris [Reaction:R-RNO-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Rattus norvegicus [Reaction:R-MMU-1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - Mus musculus
output	[SimpleEntity:R-ALL-1605658] GalNAc [lysosomal lumen] [EntityWithAccessionedSequence:R-HSA-1605614] GM2A(32-193) [lysosomal lumen] [SimpleEntity:R-ALL-1605641] GM3 [lysosomal lumen]
precedingEvent	[Reaction:R-HSA-1605717] GM2A binds and mobilizes ligands - Homo sapiens [Reaction:R-HSA-1605624] Beta-galactosidases hydrolyse mobilized GM1 to mobilized GM2 - Homo sapiens
releaseDate	2011-12-06
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:1863068] Stephan, Ralf, 2011-10-31
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-1605595
summation	[Summation:1605698] Beta-hexosaminidase A complex (bHEXA) cleaves the terminal N...
updateTrackers	[UpdateTracker:9842321] Update Tracker - [Reaction:1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - v86:[addLiteratureReference, modifyText] [UpdateTracker:9853214] Update Tracker - [Reaction:1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - v87:[modifyText]

Referrals

(updatedInstance)	[UpdateTracker:9842321] Update Tracker - [Reaction:1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - v86:[addLiteratureReference, modifyText] [UpdateTracker:9853214] Update Tracker - [Reaction:1605595] bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 - v87:[modifyText]
(precedingEvent)	[Reaction:R-HSA-1605724] NEU1,4 hydrolyze PSAP(195-273):GM3:PE [Reaction:R-HSA-1605717] GM2A binds and mobilizes ligands
(hasEvent)	[Pathway:R-HSA-9840310] Glycosphingolipid catabolism

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