KEGG   DRUG: Alglucosidase alfa
Entry
D03207                      Drug                                   
Name
Alglucosidase alfa (USAN/INN);
Alglucosidase alfa (genetical recombination) (JAN);
Lumizyme (TN);
Myozyme (TN)
Product
LUMIZYME (Genzyme Corporation)
Formula
C4758H7262N1274O1369S35
Exact mass
105270.8023
Mol weight
105336.62
Sequence
MGVRHPPCSH RLLAVCALVS LATAALLGHI LLHDFLLVPR ELSGSSPVLE ETHPAHQQGA
SRPGPRDAQA HPGRPRAVPT QCDVPPNSRF DCAPDKAITQ EQCEARGCCY IPAKQGLQGA
QMGQPWCFFP PSYPSYKLEN LSSSEMGYTA TLTRTTPTFF PKDILTLRLD VMMETENRLH
FTIKDPANRR YEVPLETPRV HSRAPSPLYS VEFSEEPFGV IVHRQLDGRV LLNTTVAPLF
FADQFLQLST SLPSQYITGL AEHLSPLMLS TSWTRITLWN RDLAPTPGAN LYGSHPFYLA
LEDGGSAHGV FLLNSNAMDV VLQPSPALSW RSTGGILDVY IFLGPEPKSV VQQYLDVVGY
PFMPPYWGLG FHLCRWGYSS TAITRQVVEN MTRAHFPLDV QWNDLDYMDS RRDFTFNKDG
FRDFPAMVQE LHQGGRRYMM IVDPAISSSG PAGSYRPYDE GLRRGVFITN ETGQPLIGKV
WPGSTAFPDF TNPTALAWWE DMVAEFHDQV PFDGMWIDMN EPSNFIRGSE DGCPNNELEN
PPYVPGVVGG TLQAATICAS SHQFLSTHYN LHNLYGLTEA IASHRALVKA RGTRPFVISR
STFAGHGRYA GHWTGDVWSS WEQLASSVPE ILQFNLLGVP LVGADVCGFL GNTSEELCVR
WTQLGAFYPF MRNHNSLLSL PQEPYSFSEP AQQAMRKALT LRYALLPHLY TLFHQAHVAG
ETVARPLFLE FPKDSSTWTV DHQLLWGEAL LITPVLQAGK AEVTGYFPLG TWYDLQTVPI
EALGSLPPPP AAPREPAIHS EGQWVTLPAP LDTINVHLRA GYIIPLQGPG LTTTESRQQP
MALAVALTKG GEARGELFWD DGESLEVLER GAYTQVIFLA RNNTIVNELV RVTSEGAGLQ
LQKVTVLGVA TAPQQVLSNG VPVSNFTYSP DTKVLDICVS LLMGEQFLVS WC
(Glycosylation sites: 140, 233, 390, 652, 882, 925)
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB07
Product: D03207<JP/US>
Efficacy
Lysosomal storage disease treatment, Enzyme replacement (alpha-glucosidase)
  Disease
Pompe disease [DS:H01940]
  Type
Enzyme replacement therapy product
Target
GAA* [HSA_VAR:2548v1] [HSA:2548] [KO:K12316]
  Pathway
hsa00052  Galactose metabolism
hsa00500  Starch and sucrose metabolism
hsa04142  Lysosome
  Network
nt06017  Glycogen metabolism
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
     A16AB07 Alglucosidase alfa
      D03207  Alglucosidase alfa (USAN/INN) <JP/US>
USP drug classification [BR:br08302]
 Genetic, Enzyme, or Protein Disorder: Replacement, Modifiers, Treatment
  Alglucosidase Alfa
   D03207  Alglucosidase alfa (USAN/INN)
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D03207  Alglucosidase alfa (USAN/INN); Alglucosidase alfa (genetical recombination) (JAN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Glycosidases
    GAA* [HSA_VAR:2548v1]
     D03207  Alglucosidase alfa (USAN/INN) <JP/US>
New drug approvals in the USA [br08319.html]
 New molecular entities and new therapeutic biological products
  D03207
New drug approvals in Europe [br08329.html]
 European public assessment reports (EPAR) authorised medicine
  D03207
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D03207
New drug approvals in the USA, Europe and Japan [br08328.html]
 Approval dates by FDA, EMA and PMDA
  D03207
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D03207
Other DBs
CAS: 420784-05-0
PubChem: 17397361
LinkDB

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KEGG   DRUG: Avalglucosidase alfa
Entry
D11744                      Drug                                   
Name
Avalglucosidase alfa (USAN/INN);
Avalglucosidase alfa (genetical recombination) (JAN);
Avalglucosidase alfa-ngpt;
Nexviazyme (TN)
Product
NEXVIAZYME (Genzyme Corporation)
Formula
C4490H6818N1197O1299S32
Exact mass
99313.5310
Mol weight
99375.55
Sequence
QQGASRPGPR DAQAHPGRPR AVPTQCDVPP NSRFDCAPDK AITQEQCEAR GCCYIPAKQG
LQGAQMGQPW CFFPPSYPSY KLENLSSSEM GYTATLTRTT PTFFPKDILT LRLDVMMETE
NRLHFTIKDP ANRRYEVPLE TPRVHSRAPS PLYSVEFSEE PFGVIVHRQL DGRVLLNTTV
APLFFADQFL QLSTSLPSQY ITGLAEHLSP LMLSTSWTRI TLWNRDLAPT PGANLYGSHP
FYLALEDGGS AHGVFLLNSN AMDVVLQPSP ALSWRSTGGI LDVYIFLGPE PKSVVQQYLD
VVGYPFMPPY WGLGFHLCRW GYSSTAITRQ VVENMTRAHF PLDVQWNDLD YMDSRRDFTF
NKDGFRDFPA MVQELHQGGR RYMMIVDPAI SSSGPAGSYR PYDEGLRRGV FITNETGQPL
IGKVWPGSTA FPDFTNPTAL AWWEDMVAEF HDQVPFDGMW IDMNEPSNFI RGSEDGCPNN
ELENPPYVPG VVGGTLQAAT ICASSHQFLS THYNLHNLYG LTEAIASHRA LVKARGTRPF
VISRSTFAGH GRYAGHWTGD VWSSWEQLAS SVPEILQFNL LGVPLVGADV CGFLGNTSEE
LCVRWTQLGA FYPFMRNHNS LLSLPQEPYS FSEPAQQAMR KALTLRYALL PHLYTLFHQA
HVAGETVARP LFLEFPKDSS TWTVDHQLLW GEALLITPVL QAGKAEVTGY FPLGTWYDLQ
TVPIEALGSL PPPPAAPREP AIHSEGQWVT LPAPLDTINV HLRAGYIIPL QGPGLTTTES
RQQPMALAVA LTKGGEARGE LFWDDGESLE VLERGAYTQV IFLARNNTIV NELVRVTSEG
AGLQLQKVTV LGVATAPQQV LSNGVPVSNF TYSPDTKVLD ICVSLLMGEQ FLVSWC
(Disulfide bridge:26-53, 36-52, 47-71, 477-502, 591-602, 882-896)
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB22
Product: D11744<JP/US>
Efficacy
Enzyme replacement (alpha-glucosidase)
  Disease
Pompe disease [DS:H01940]
  Type
Enzyme replacement therapy product
Comment
Treatment of Pompe disease
Target
GAA* [HSA_VAR:2548v1] [HSA:2548] [KO:K12316]
  Pathway
hsa00052  Galactose metabolism
hsa00500  Starch and sucrose metabolism
hsa04142  Lysosome
  Network
nt06017  Glycogen metabolism
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
     A16AB22 Avalglucosidase alfa
      D11744  Avalglucosidase alfa (USAN/INN) <JP/US>
USP drug classification [BR:br08302]
 Genetic, Enzyme, or Protein Disorder: Replacement, Modifiers, Treatment
  Avalglucosidase Alfa
   D11744  Avalglucosidase alfa (USAN/INN)
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D11744  Avalglucosidase alfa (USAN/INN); Avalglucosidase alfa (genetical recombination) (JAN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Glycosidases
    GAA* [HSA_VAR:2548v1]
     D11744  Avalglucosidase alfa (USAN/INN) <JP/US>
New drug approvals in the USA [br08319.html]
 New molecular entities and new therapeutic biological products
  D11744
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D11744
New drug approvals in the USA, Europe and Japan [br08328.html]
 Approval dates by FDA, EMA and PMDA
  D11744
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D11744
Other DBs
CAS: 1802558-87-7
PubChem: 405226610
LinkDB

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KEGG   DRUG: Cipaglucosidase alfa
Entry
D11798                      Drug                                   
Name
Cipaglucosidase alfa (USAN);
Cipaglucosidase alfa (genetical recombination) (JAN);
Cipaglucosidase alfa-atga;
Pombiliti (TN)
Product
POMBILITI (AMICUS THERAPEUTICS US)
Formula
C4489H6817N1197O1298S32
Exact mass
99284.5283
Mol weight
99346.54
Sequence
QQGASRPGPR DAQAHPGRPR AVPTQCDVPP NSRFDCAPDK AITQEQCEAR GCCYIPAKQG
LQGAQMGQPW CFFPPSYPSY KLENLSSSEM GYTATLTRTT PTFFPKDILT LRLDVMMETE
NRLHFTIKDP ANRRYEVPLE TPHVHSRAPS PLYSVEFSEE PFGVIVRRQL DGRVLLNTTV
APLFFADQFL QLSTSLPSQY ITGLAEHLSP LMLSTSWTRI TLWNRDLAPT PGANLYGSHP
FYLALEDGGS AHGVFLLNSN AMDVVLQPSP ALSWRSTGGI LDVYIFLGPE PKSVVQQYLD
VVGYPFMPPY WGLGFHLCRW GYSSTAITRQ VVENMTRAHF PLDVQWNDLD YMDSRRDFTF
NKDGFRDFPA MVQELHQGGR RYMMIVDPAI SSSGPAGSYR PYDEGLRRGV FITNETGQPL
IGKVWPGSTA FPDFTNPTAL AWWEDMVAEF HDQVPFDGMW IDMNEPSNFI RGSEDGCPNN
ELENPPYVPG VVGGTLQAAT ICASSHQFLS THYNLHNLYG LTEAIASHRA LVKARGTRPF
VISRSTFAGH GRYAGHWTGD VWSSWEQLAS SVPEILQFNL LGVPLVGADV CGFLGNTSEE
LCVRWTQLGA FYPFMRNHNS LLSLPQEPYS FSEPAQQAMR KALTLRYALL PHLYTLFHQA
HVAGETVARP LFLEFPKDSS TWTVDHQLLW GEALLITPVL QAGKAEVTGY FPLGTWYDLQ
TVPVEALGSL PPPPAAPREP AIHSEGQWVT LPAPLDTINV HLRAGYIIPL QGPGLTTTES
RQQPMALAVA LTKGGEARGE LFWDDGESLE VLERGAYTQV IFLARNNTIV NELVRVTSEG
AGLQLQKVTV LGVATAPQQV LSNGVPVSNF TYSPDTKVLD ICVSLLMGEQ FLVSWC
(Disulfide bridge: 26-53, 36-52, 47-71, 477-502, 591-602)
  Type
Peptide
Remark
ATC code: A16AB23
Product: D11798<US>
Efficacy
Enzyme replacement (alpha-glucosidase)
  Disease
Pompe disease [DS:H01940]
  Type
Enzyme replacement therapy product
Comment
Treatment of Pompe disease
Target
GAA* [HSA_VAR:2548v1] [HSA:2548] [KO:K12316]
  Pathway
hsa00052  Galactose metabolism
hsa00500  Starch and sucrose metabolism
hsa04142  Lysosome
  Network
nt06017  Glycogen metabolism
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
     A16AB23 Cipaglucosidase alfa
      D11798  Cipaglucosidase alfa (USAN) <US>
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Glycosidases
    GAA* [HSA_VAR:2548v1]
     D11798  Cipaglucosidase alfa (USAN) <US>
New drug approvals in the USA [br08319.html]
 New molecular entities and new therapeutic biological products
  D11798
New drug approvals in Europe [br08329.html]
 European public assessment reports (EPAR) authorised medicine
  D11798
New drug approvals in the USA, Europe and Japan [br08328.html]
 Approval dates by FDA, EMA and PMDA
  D11798
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D11798
Other DBs
CAS: 2359727-71-0
PubChem: 405226664
LinkDB

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