KEGG DRUG: Avalglucosidase alfa

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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