KEGG   DRUG: Idursulfase
Entry
D04499                      Drug                                   
Name
Idursulfase (USAN/INN);
Idursulfase (genetical recombination) (JAN);
Elaprase (TN)
Product
ELAPRASE (Takeda Pharmaceuticals America)
Sequence
SETQANSTTD ALNVLLIIVD DLRPSLGCYG DKLVRSPNID QLASHSLLFQ NAFAQQAVCA
PSRVSFLTGR RPDTTRLYDF NSYWRVHAGN FSTIPQYFKE NGYVTMSVGK VFHPGISSNH
TDDSPYSWSF PPYHPSSEKY ENTKTCRGPD GELHANLLCP VDVLDVPEGT LPDKQSTEQA
IQLLEKMKTS ASPFFLAVGY HKPHIPFRYP KEFQKLYPLE NITLAPDPEV PDGLPPVAYN
PWMDIRQRED VQALNISVPY GPIPVDFQRK IRQSYFASVS YLDTQVGRLL SALDDLQLAN
STIIAFTSDH GWALGEHGEW AKYSNFDVAT HVPLIFYVPG RTASLPEAGE KLFPYLDPFD
SASQLMEPGR QSMDLVELVS LFPTLAGLAG LQVPPRCPVP SFHVELCREG KNLLKHFRFR
DLEEDPYLPG NPRELIAYSQ YPRPSDIPQW NSDKPSLKDI KIMGYSIRTI DYRYTVWVGF
NPDEFLANFS DIHAGELYFV DSDPLQDHNM YNDSQGGDLF QLLMP
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB09
Product: D04499<JP/US>
Efficacy
Lysosomal storage disease treatment, Enzyme replacement (iduronate-2-sulfatase)
  Disease
Hunter syndrome [DS:H00129]
  Type
Enzyme replacement therapy product
Target
IDS* [HSA_VAR:3423v1] [HSA:3423] [KO:K01136]
  Pathway
hsa00531  Glycosaminoglycan degradation
hsa04142  Lysosome
  Network
nt06012  Glycosaminoglycan 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
     A16AB09 Idursulfase
      D04499  Idursulfase (USAN/INN) <JP/US>
USP drug classification [BR:br08302]
 Genetic, Enzyme, or Protein Disorder: Replacement, Modifiers, Treatment
  Idursulfase
   D04499  Idursulfase (USAN/INN)
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D04499  Idursulfase (USAN/INN); Idursulfase (genetical recombination) (JAN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Sulfatases
    IDS* [HSA_VAR:3423v1]
     D04499  Idursulfase (USAN/INN) <JP/US>
New drug approvals in the USA [br08319.html]
 New molecular entities and new therapeutic biological products
  D04499
New drug approvals in Europe [br08329.html]
 European public assessment reports (EPAR) authorised medicine
  D04499
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D04499
New drug approvals in the USA, Europe and Japan [br08328.html]
 Approval dates by FDA, EMA and PMDA
  D04499
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D04499
Other DBs
CAS: 50936-59-9
PubChem: 47206347
LinkDB

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KEGG   DRUG: Idursulfase beta
Entry
D11025                      Drug                                   
Name
Idursulfase beta (INN);
Idursulfase beta (genetical recombination) (JAN);
Hunterase (TN)
Formula
C2689H4051N699O793S13
Exact mass
59238.4521
Mol weight
59274.9897
Sequence
SETQANSTTD ALNVLLIIVD DLRPSLGCYG DKLVRSPNID QLASHSLLFQ NAFAQQAVCA
PSRVSFLTGR RPDTTRLYDF NSYWRVHAGN FSTIPQYFKE NGYVTMSVGK VFHPGISSNH
TDDSPYSWSF PPYHPSSEKY ENTKTCRGPD GELHANLLCP VDVLDVPEGT LPDKQSTEQA
IQLLEKMKTS ASPFFLAVGY HKPHIPFRYP KEFQKLYPLE NITLAPDPEV PDGLPPVAYN
PWMDIRQRED VQALNISVPY GPIPVDFQRK IRQSYFASVS YLDTQVGRLL SALDDLQLAN
STIIAFTSDH GWALGEHGEW AKYSNFDVAT HVPLIFYVPG RTASLPEAGE KLFPYLDPFD
SASQLMEPGR QSMDLVELVS LFPTLAGLAG LQVPPRCPVP SFHVELCREG KNLLKHFRFR
DLEEDPYLPG NPRELIAYSQ YPRPSDIPQW NSDKPSLKDI KIMGYSIRTI DYRYTVWVGF
NPDEFLANFS DIHAGELYFV DSDPLQDHNM YNDSQGGDLF QLLMP
(Disulfide bridge: 146-159, 397-407)
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB16
Product: D11025<JP>
Efficacy
Lysosomal storage disease treatment, Enzyme replacement (iduronate-2-sulfatase)
  Type
Enzyme replacement therapy product
Comment
Treatment of Hunter syndrome
Target
IDS* [HSA_VAR:3423v1] [HSA:3423] [KO:K01136]
  Pathway
hsa00531  Glycosaminoglycan degradation
hsa04142  Lysosome
  Network
nt06012  Glycosaminoglycan 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
     A16AB16 Idursulfase beta
      D11025  Idursulfase beta (INN) <JP>
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D11025  Idursulfase beta (INN); Idursulfase beta (genetical recombination) (JAN)
Target-based classification of drugs [BR:br08310]
 Enzymes
  Hydrolases (EC3)
   Sulfatases
    IDS* [HSA_VAR:3423v1]
     D11025  Idursulfase beta (INN) <JP>
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D11025
Pharmacogenomic biomarkers [br08341.html]
 Germline mutations in genetic disorder treatments
  D11025
Other DBs
CAS: 1271734-34-9
PubChem: 350078344
LinkDB

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KEGG   DRUG: Pabinafusp alfa
Entry
D11812                      Drug                                   
Name
Pabinafusp alfa (genetical recombination) (JAN);
Pabinafusp alfa (INN);
Izcargo (TN)
Sequence
(Heavy chain)
EVQLVQSGAE VKKPGESLKI SCKGSGYSFT NYWLGWVRQM PGKGLEWMGD IYPGGDYPTY
SEKFKVQVTI SADKSISTAY LQWSSLKASD TAMYYCARSG NYDEVAYWGQ GTLVTVSSAS
TKGPSVFPLA PSSKSTSGGT AALGCLVKDY FPEPVTVSWN SGALTSGVHT FPAVLQSSGL
YSLSSVVTVP SSSLGTQTYI CNVNHKPSNT KVDKKVEPKS CDKTHTCPPC PAPELLGGPS
VFLFPPKPKD TLMISRTPEV TCVVVDVSHE DPEVKFNWYV DGVEVHNAKT KPREEQYNST
YRVVSVLTVL HQDWLNGKEY KCKVSNKALP APIEKTISKA KGQPREPQVY TLPPSRDELT
KNQVSLTCLV KGFYPSDIAV EWESNGQPEN NYKTTPPVLD SDGSFFLYSK LTVDKSRWQQ
GNVFSCSVMH EALHNHYTQK SLSLSPGKGS SETQANSTTD ALNVLLIIVD DLRPSLGCYG
DKLVRSPNID QLASHSLLFQ NAFAQQAVCA PSRVSFLTGR RPDTTRLYDF NSYWRVHAGN
FSTIPQYFKE NGYVTMSVGK VFHPGISSNH TDDSPYSWSF PPYHPSSEKY ENTKTCRGPD
GELHANLLCP VDVLDVPEGT LPDKQSTEQA IQLLEKMKTS ASPFFLAVGY HKPHIPFRYP
KEFQKLYPLE NITLAPDPEV PDGLPPVAYN PWMDIRQRED VQALNISVPY GPIPVDFQRK
IRQSYFASVS YLDTQVGRLL SALDDLQLAN STIIAFTSDH GWALGEHGEW AKYSNFDVAT
HVPLIFYVPG RTASLPEAGE KLFPYLDPFD SASQLMEPGR QSMDLVELVS LFPTLAGLAG
LQVPPRCPVP SFHVELCREG KNLLKHFRFR DLEEDPYLPG NPRELIAYSQ YPRPSDIPQW
NSDKPSLKDI KIMGYSIRTI DYRYTVWVGF NPDEFLANFS DIHAGELYFV DSDPLQDHNM
YNDSQGGDLF QLLMP
(Light chain)
DIVMTQTPLS LSVTPGQPAS ISCRSSQSLV HSNGNTYLHW YLQKPGQSPQ LLIYKVSNRF
SGVPDRFSGS GSGTDFTLKI SRVEAEDVGV YYCSQSTHVP WTFGQGTKVE IKRTVAAPSV
FIFPPSDEQL KSGTASVVCL LNNFYPREAK VQWKVDNALQ SGNSQESVTE QDSKDSTYSL
SSTLTLSKAD YEKHKVYACE VTHQGLSSPV TKSFNRGEC
(Disulfide bridge: H22-H96, H145-H201, H221-L219, H227-H'227, H230-H'230, H262-H322, H368-H426, H596-H609, H847-H857, H'22-H'96, H'145-H'201, H'221-L'219, H'262-H'322, H'368-H'426, H'596-H'609, H'847-H'857, L23-L93, L139-L199, L'23-L'93, L'139-L'199)
  Type
Peptide
Remark
Therapeutic category: 3959
ATC code: A16AB27
Product: D11812<JP>
Efficacy
Inherited metabolic disorder therapeutic agent
Comment
Fusion protein, enzyme-antibody
Target
IDS* [HSA_VAR:3423v1] [HSA:3423] [KO:K01136]
TFRC (CD71) [HSA:7037] [KO:K06503]
  Pathway
hsa00531  Glycosaminoglycan degradation
hsa04066  HIF-1 signaling pathway
hsa04142  Lysosome
hsa04144  Endocytosis
hsa04145  Phagosome
hsa04216  Ferroptosis
hsa04640  Hematopoietic cell lineage
  Network
nt06012  Glycosaminoglycan 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
     A16AB27 Pabinafusp alfa
      D11812  Pabinafusp alfa (genetical recombination) (JAN) <JP>
Therapeutic category of drugs in Japan [BR:br08301]
 3  Agents affecting metabolism
  39  Other agents affecting metabolism
   395  Enzyme preparations
    3959  Others
     D11812  Pabinafusp alfa (genetical recombination) (JAN); Pabinafusp alfa (INN)
Target-based classification of drugs [BR:br08310]
 Cell surface molecules and ligands
  Other cell surface molecules
   CD molecules
    TFRC (CD71)
     D11812  Pabinafusp alfa (genetical recombination) (JAN) <JP>
 Enzymes
  Hydrolases (EC3)
   Sulfatases
    IDS* [HSA_VAR:3423v1]
     D11812  Pabinafusp alfa (genetical recombination) (JAN) <JP>
New drug approvals in Japan [br08318.html]
 Drugs with new active ingredients
  D11812
New drug approvals in the USA, Europe and Japan [br08328.html]
 Approval dates by FDA, EMA and PMDA
  D11812
Other DBs
CAS: 2140211-48-7
PubChem: 405226677
LinkDB

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