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Mature HEXA [lysosomal lumen]
Stable Identifier
R-HSA-9842391
Type
Complex
Species
Homo sapiens
Compartment
lysosomal lumen
Synonyms
Mature beta-hexosaminidase subunit alpha
Locations in the PathwayBrowser
Expand all
Metabolism (Homo sapiens)
Metabolism of carbohydrates (Homo sapiens)
Glycosaminoglycan metabolism (Homo sapiens)
Chondroitin sulfate/dermatan sulfate metabolism (Homo sapiens)
CS/DS degradation (Homo sapiens)
HEXA cleaves the terminal GalNAc from DS (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
Hyaluronan metabolism (Homo sapiens)
Hyaluronan uptake and degradation (Homo sapiens)
HEXA cleaves the terminal GalNAc from small HA fragments (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
Keratan sulfate/keratin metabolism (Homo sapiens)
Keratan sulfate degradation (Homo sapiens)
HEXA cleaves the terminal GalNAc from keratan sulfate (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
Metabolism of lipids (Homo sapiens)
Sphingolipid metabolism (Homo sapiens)
Glycosphingolipid metabolism (Homo sapiens)
Glycosphingolipid catabolism (Homo sapiens)
bHEXA hydrolyzes GM2A:GM2 to GM2A:GM3 (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
bHEXA,bHEXB hydrolyze GM2A:GA2 to GM2A:LacCer (Homo sapiens)
bHEXA,bHEXB [lysosomal lumen] (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
bHEXA,bHEXB hydrolyze PSAP(195-273):Gb4Cer:PE (Homo sapiens)
bHEXA,bHEXB [lysosomal lumen] (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
bHEXA,bHEXS hydrolyze GM2A:SM2 (Homo sapiens)
bHEXA,bHEXS [lysosomal lumen] (Homo sapiens)
bHEXA [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
bHEXS [lysosomal lumen] (Homo sapiens)
HEXA dimer [lysosomal lumen] (Homo sapiens)
Mature HEXA [lysosomal lumen] (Homo sapiens)
Literature References
PubMed ID
Title
Journal
Year
16698036
Crystallographic structure of human beta-hexosaminidase A: interpretation of Tay-Sachs mutations and loss of GM2 ganglioside hydrolysis
Mahuran, DJ
,
James, MN
,
Lemieux, MJ
,
Mark, BL
,
Withers, SG
,
Cherney, MM
J Mol Biol
2006
Participants
components
HEXA(23-73) [lysosomal lumen]
(Homo sapiens)
HEXA(88-528) [lysosomal lumen]
(Homo sapiens)
Participates
as a component of
HEXA dimer [lysosomal lumen] (Homo sapiens)
Inferred To
Mature HEXA [lysosomal lumen]
(Bos taurus)
Mature HEXA [lysosomal lumen]
(Caenorhabditis elegans)
Mature HEXA [lysosomal lumen]
(Canis familiaris)
Mature HEXA [lysosomal lumen]
(Dictyostelium discoideum)
Mature HEXA [lysosomal lumen]
(Drosophila melanogaster)
Mature HEXA [lysosomal lumen]
(Gallus gallus)
Mature HEXA [lysosomal lumen]
(Mus musculus)
Mature HEXA [lysosomal lumen]
(Rattus norvegicus)
Mature HEXA [lysosomal lumen]
(Sus scrofa)
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