Welcome to Entry Card of Lysosomal Enzymes


Details of RareLSD ID 1034
RareLSD_Id1034
ENZYMECathepsin Z
GENECTSZ
E.C.NUMBER3.4.18.1
FAMILYPapain cysteine protease (C1)
CYTOGENETICS20q13.32
DISEASEdacryoadenitis
SNPrs1000409839,rs1000441604, ,rs1000506139,rs1000510762,rs1000664531
DEPOSITNA
REF9642240 , 18420963, 10653163
TEMP (in Celsius)37
pI5.1
pH3.5-5.5
Catalytic NucleophileCys25
Catalytic Acid/Base
H2O
Substrate(2,4-dinitrophenyl)GFFGW
Product2,4-dinitrophenyl)GFFG + LTrp
Structure(PDB/Phyre2_ID)1EF7
kM0.0018
DRUGE64c ,Odacatib (MK0822)
SEQ LENGTH303
AA SEQMARRGPGWRPLLLLVLLAGAAQGGLYFRRGQTCYRPLRGDGLAPLGRSTYPRPHEYLSPADLPKSWDWRNVDGVNYASITRNQHIPQYCGSCWAHASTSAMADRINIKRKGAWPSTLLSVQNVIDCGNAGSCEGGNDLSVWDYAHQHGIPDETCNNYQAKDQECDKFNQCGTCNEFKECHAIRNYTLWRVGDYGSLSGREKMMAEIYANGPISCGIMATERLANYTGGIYAEYQDTTYINHVVSVAGWGISDGTEYWIVRNSWGEPWGERGWLRIVTSTYKDGKGARYNLAIEEHCTFGDPIV
MODIFICATIONN-linked Glycosylation:Asn184,Asn224
INHIBITORCA074,chicken cystatin,GFGsemicarbazone,Stefin A
ACTIVE SITE RESIDUEGln83,Cys92,His241,Asn261
DISULPHIDE BRIDGES*89-132,*126-164,*154-170,*173-179,*214-296
PSEUDOGENESPGOHUM00000251120
MISCELLANEOUSNA
PARALOGSCHI3L1,CHIA,CHI3L2,OVGP1
MECHANISMRelease of Cterminal amino acid residues with broad specificity, but lacks action on Cterminal proline
INHERITANCE PATTERNNA
ORGAN AFFECTEDNA
ENZYME LOCATIONOD
UniProt IDQ9UBR2