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
99313.531
Mol weight
99375.4984
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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