Protein
Protein Name Lysosomal acid glucosylceramidase
#PDB ID 5
Organism Homo sapiens (Human)
Uniprot ID/ACC P04062 (GLCM_HUMAN)
Synonym(s)
  •   D-glucosyl-N-acylsphingosine glucohydrolase
  • SGTase
  • acid beta-glucosidase
  • alglucerase
  • beta-GC
  • beta-glucocerebrosidase
  • cholesterol glucosyltransferase
  • cholesteryl-beta-glucosidase
  • glucosidase
  • beta
  • acid
  • glucosylceramidase-like protein
  • imiglucerase
  • lysosomal acid GCase
  • lysosomal glucocerebrosidase
  • GBA1
  • GCB
  • GLUC
show 17 synonym(s)
Gene Symbol GBA
Gene ID 2629
Sequence MEFSSPSREECPKPLSRVSIMAGSLTGLLLLQAVSWASGARPCIPKSFGYSSVVCVCNATYCDSFDPPTFPALGTFSRYESTRSGRRMELSMGPIQANHTGTGLLLTLQPEQKFQKVKGFGGAMTDAAALNILALSPPAQNLLLKSYFSEEGIGYNIIRVPMASCDFSIRTYTYADTPDDFQLHNFSLPEEDTKLKIPLIHRALQLAQRPVSLLASPWTSPTWLKTNGAVNGKGSLKGQPGDIYHQTWARYFVKFLDAYAEHKLQFWAVTAENEPSAGLLSGYPFQCLGFTPEHQRDFIARDLGPTLANSTHHNVRLLMLDDQRLLLPHWAKVVLTDPEAAKYVHGIAVHWYLDFLAPAKATLGETHRLFPNTMLFASEACVGSKFWEQSVRLGSWDRGMQYSHSIITNLLYHVVGWTDWNLALNPEGGPNWVRNFVDSPIIVDITKDTFYKQPMFYHLGHFSKFIPEGSQRVGLVASQKNDLDAVALMHPDGSAVVVVLNRSSKDVPLTIKDPAVGFLETISPGYSIHTYLWRRQ
Protein Class HYDROLASE
Function Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose (PubMed:9201993, PubMed:24211208, PubMed:15916907). Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Also plays a role in cholesterol metabolism (PubMed:24211208, PubMed:26724485). May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol (PubMed:24211208, PubMed:26724485). The short chain saturated C8:0-GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide (PubMed:26724485). Finally, may also hydrolyze cholesteryl-beta-D-glucoside to produce D-glucose and cholesterol (PubMed:24211208, PubMed:26724485).
Bound Ligands
Ligand ID HET Code PDB ID Binding Affinity Binding Database Structure Complex Card
COVPDB908 HK2 6Q6N
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SHOW
COVPDB1282 RJR 6Q6L
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SHOW
COVPDB627 INS 1Y7V
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SHOW
COVPDB911 HKW 6Q6K
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SHOW
COVPDB627 CBU 2VT0
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SHOW