KEGG   DRUG: Agalsidase alfa
Entry
D02784                      Drug                                   
Name
Agalsidase alfa (USAN/INN);
Agalsidase alfa (genetical recombination) (JAN)
Sequence
LDNGLARTPT MGWLHWERFM CNLDCQEEPD SCISEKLFME MAELMVSEGW KDAGYEYLCI
DDCWMAPQRD SEGRLQADPQ RFPHGIRQLA NYVHSKGLKL GIYADVGNKT CAGFPGSFGY
YDIDAQTFAD WGVDLLKFDG CYCDSLENLA DGYKHMSLAL NRTGRSIVYS CEWPLYMWPF
QKPNYTEIRQ YCNHWRNFAD IDDSWKSIKS ILDWTSFNQE RIVDVAGPGG WNDPDMLVIG
NFGLSWNQQV TQMALWAIMA APLFMSNDLR HISPQAKALL QDKDVIAINQ DPLGKQGYQL
RQGDNFEVWE RPLSGLAWAV AMINRQEIGG PRSYTIAVAS LGKGVACNPA CFITQLLPVK
RKLGFYEWTS RLRSHINPTG TVLLQLENTM QMSLKDLL
(Glycosylation site: 108; 161; 184)
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB03
Product: D02784<JP>
Efficacy
Lysosomal storage disease treatment, Enzyme replacement (alpha-galactosidase)
  Disease
Fabry disease [DS:H00125]
  Type
Enzyme replacement therapy product
Target
GLA* [HSA_VAR:2717v1] [HSA:2717] [KO:K01189]
  Pathway
hsa00600  Sphingolipid metabolism
hsa04142  Lysosome
  Network
nt06014  Sphingolipid degradation
Interaction
Brite
Anatomical Therapeutic Chemical (ATC) classification [BR:br08303]
 A ALIMENTARY TRACT AND METABOLISM
  A16 OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
   A16A OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
    A16AB Enzymes
     A16AB03 Agalsidase alfa
      D02784  Agalsidase alfa (USAN/INN) <JP>
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D02784  Agalsidase alfa (USAN/INN); Agalsidase alfa (genetical recombination) (JAN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Glycosidases
    GLA* [HSA_VAR:2717v1]
     D02784  Agalsidase alfa (USAN/INN) <JP>
New drug approvals in Europe [br08329.html]
 European public assessment reports (EPAR) authorised medicine
  D02784
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D02784
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D02784
Other DBs
CAS: 104138-64-9
PubChem: 17396942
LinkDB

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KEGG   DRUG: Agalsidase beta
Entry
D03228                      Drug                                   
Name
Agalsidase beta (INN);
Agalsidase beta (genetical recombination) (JAN);
Agalsidase beta (genetical recombination) [Agalsidase beta biosimilar 1] (JAN);
Fabrazyme (TN)
Product
FABRAZYME (Genzyme Corporation)
Sequence
LDNGLARTPT MGWLHWERFM CNLDCQEEPD SCISEKLFME MAELMVSEGW KDAGYEYLCI
DDCWMAPQRD SEGRLQADPQ RFPHGIRQLA NYVHSKGLKL GIYADVGNKT CAGFPGSFGY
YDIDAQTFAD WGVDLLKFDG CYCDSLENLA DGYKHMSLAL NRTGRSIVYS CEWPLYMWPF
QKPNYTEIRQ YCNHWRNFAD IDDSWKSIKS ILDWTSFNQE RIVDVAGPGG WNDPDMLVIG
NFGLSWNQQV TQMALWAIMA APLFMSNDLR HISPQAKALL QDKDVIAINQ DPLGKQGYQL
RQGDNFEVWE RPLSGLAWAV AMINRQEIGG PRSYTIAVAS LGKGVACNPA CFITQLLPVK
RKLGFYEWTS RLRSHINPTG TVLLQLENTM QMSLKDLL
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB04
Product: D03228<JP/US>
Efficacy
Lysosomal storage disease treatment, Enzyme replacement (alpha-galactosidase)
  Disease
Fabry disease [DS:H00125]
  Type
Enzyme replacement therapy product
Target
GLA* [HSA_VAR:2717v1] [HSA:2717] [KO:K01189]
  Pathway
hsa00600  Sphingolipid metabolism
hsa04142  Lysosome
  Network
nt06014  Sphingolipid degradation
Interaction
Brite
Anatomical Therapeutic Chemical (ATC) classification [BR:br08303]
 A ALIMENTARY TRACT AND METABOLISM
  A16 OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
   A16A OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
    A16AB Enzymes
     A16AB04 Agalsidase beta
      D03228  Agalsidase beta (INN) <JP/US>
USP drug classification [BR:br08302]
 Genetic, Enzyme, or Protein Disorder: Replacement, Modifiers, Treatment
  Agalsidase Beta
   D03228  Agalsidase beta (INN)
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D03228  Agalsidase beta (INN); Agalsidase beta (genetical recombination) (JAN); Agalsidase beta (genetical recombination) [Agalsidase beta biosimilar 1] (JAN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Glycosidases
    GLA* [HSA_VAR:2717v1]
     D03228  Agalsidase beta (INN) <JP/US>
New drug approvals in Europe [br08329.html]
 European public assessment reports (EPAR) authorised medicine
  D03228
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D03228
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D03228
Other DBs
CAS: 104138-64-9
PubChem: 17397381
LinkDB

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KEGG   DRUG: Pegunigalsidase alfa
Entry
D11685                      Drug                                   
Name
Pegunigalsidase alfa (USAN/INN);
Pegunigalsidase alfa-iwxj;
Elfabrio (TN)
Product
ELFABRIO (Chiesi USA)
Formula
C2060H3130N552O601S27
Exact mass
46080.3788
Mol weight
46110.04
Sequence
GLDNGLARTP TMGWLHWERF MCNLDCQEEP DSCISEKLFM EMAELMVSEG WKDAGYEYLC
IDDCWMAPQR DSEGRLQADP QRFPHGIRQL ANYVHSKGLK LGIYADVGNK TCAGFPGSFG
YYDIDAQTFA DWGVDLLKFD GCYCDSLENL ADGYKHMSLA LNRTGRSIVY SCEWPLYMWP
FQKPNYTEIR QYCNHWRNFA DIDDSWKSIK SILDWTSFNQ ERIVDVAGPG GWNDPDMLVI
GNFGLSWNQQ VTQMALWAIM AAPLFMSNDL RHISPQAKAL LQDKDVIAIN QDPLGKQGYQ
LRQGDNFEVW ERPLSGLAWA VAMINRQEIG GPRSYTIAVA SLGKGVACNP ACFITQLLPV
KRKLGFYEWT SRLRSHINPT GTVLLQLENT MQMSLKDLLS EKDEL
  Type
Peptide
Remark
ATC code: A16AB20
Product: D11685<US>
Efficacy
Lysosomal storage disease treatment, Enzyme replacement (alpha-galactosidase)
  Disease
Fabry disease [DS:H00125]
  Type
Enzyme replacement therapy product
Comment
Treatment of Fabry disease
Target
GLA* [HSA_VAR:2717v1] [HSA:2717] [KO:K01189]
  Pathway
hsa00600  Sphingolipid metabolism
hsa04142  Lysosome
  Network
nt06014  Sphingolipid degradation
Brite
Anatomical Therapeutic Chemical (ATC) classification [BR:br08303]
 A ALIMENTARY TRACT AND METABOLISM
  A16 OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
   A16A OTHER ALIMENTARY TRACT AND METABOLISM PRODUCTS
    A16AB Enzymes
     A16AB20 Pegunigalsidase alfa
      D11685  Pegunigalsidase alfa (USAN/INN) <US>
USP drug classification [BR:br08302]
 Genetic, Enzyme, or Protein Disorder: Replacement, Modifiers, Treatment
  Pegunigalsidase Alfa
   D11685  Pegunigalsidase alfa (USAN/INN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Glycosidases
    GLA* [HSA_VAR:2717v1]
     D11685  Pegunigalsidase alfa (USAN/INN) <US>
New drug approvals in the USA [br08319.html]
 New molecular entities and new therapeutic biological products
  D11685
New drug approvals in Europe [br08329.html]
 European public assessment reports (EPAR) authorised medicine
  D11685
New drug approvals in the USA, Europe and Japan [br08328.html]
 Approval dates by FDA, EMA and PMDA
  D11685
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D11685
Other DBs
CAS: 1644392-61-9
PubChem: 405226551
LinkDB

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