BRENDA - Enzyme Database show
show all sequences of 3.5.1.26

Functional analyses of active site residues of human lysosomal aspartylglucosaminidase: implications for catalytic mechanism and autocatalytic activation

Tikkanen, R.; Riikonen, A.; Oinonen, C.; Rouvinen, J.; Peltonen, L.; EMBO J. 15, 2954-2960 (1996)

Data extracted from this reference:

Cloned(Commentary)
Commentary
Organism
expression in COS cells
Homo sapiens
Engineering
Amino acid exchange
Commentary
Organism
R234A
nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
R234L
nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
R234Q
nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
T224A
nearly complete decrease of activity
Homo sapiens
T224S
nearly complete decrease of activity
Homo sapiens
T257A
nearly complete decrease of activity; nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
KM Value [mM]
KM Value [mM]
KM Value Maximum [mM]
Substrate
Commentary
Organism
Structure
2.5
-
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine
-
Homo sapiens
Localization
Localization
Commentary
Organism
GeneOntology No.
Textmining
lysosome
-
Homo sapiens
5764
-
Natural Substrates/ Products (Substrates)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O
Homo sapiens
involved in degradation of glycoproteins
2-acetamido-1-amino-1,2-dideoxy-beta-D-glucose + L-aspartate
-
Homo sapiens
?
Organism
Organism
Primary Accession No. (UniProt)
Commentary
Textmining
Homo sapiens
-
-
-
Purification (Commentary)
Commentary
Organism
to homogeneity, chromatography techniques
Homo sapiens
Source Tissue
Source Tissue
Commentary
Organism
Textmining
leukocyte
-
Homo sapiens
-
placenta
-
Homo sapiens
-
Substrates and Products (Substrate)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O
involved in degradation of glycoproteins
209051
Homo sapiens
2-acetamido-1-amino-1,2-dideoxy-beta-D-glucose + L-aspartate
-
209051
Homo sapiens
?
Cloned(Commentary) (protein specific)
Commentary
Organism
expression in COS cells
Homo sapiens
Engineering (protein specific)
Amino acid exchange
Commentary
Organism
R234A
nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
R234L
nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
R234Q
nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
T224A
nearly complete decrease of activity
Homo sapiens
T224S
nearly complete decrease of activity
Homo sapiens
T257A
nearly complete decrease of activity; nearly complete decrease of activity, misprocessing of precursor in ER
Homo sapiens
KM Value [mM] (protein specific)
KM Value [mM]
KM Value Maximum [mM]
Substrate
Commentary
Organism
Structure
2.5
-
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine
-
Homo sapiens
Localization (protein specific)
Localization
Commentary
Organism
GeneOntology No.
Textmining
lysosome
-
Homo sapiens
5764
-
Natural Substrates/ Products (Substrates) (protein specific)
Natural Substrates
Organism
Commentary (Nat. Sub.)
Natural Products
Commentary (Nat. Pro.)
Organism (Nat. Pro.)
Reversibility
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O
Homo sapiens
involved in degradation of glycoproteins
2-acetamido-1-amino-1,2-dideoxy-beta-D-glucose + L-aspartate
-
Homo sapiens
?
Purification (Commentary) (protein specific)
Commentary
Organism
to homogeneity, chromatography techniques
Homo sapiens
Source Tissue (protein specific)
Source Tissue
Commentary
Organism
Textmining
leukocyte
-
Homo sapiens
-
placenta
-
Homo sapiens
-
Substrates and Products (Substrate) (protein specific)
Substrates
Commentary Substrates
Literature (Substrates)
Organism
Products
Commentary (Products)
Literature (Products)
Organism (Products)
Reversibility
N4-(beta-N-acetyl-D-glucosaminyl)-L-asparagine + H2O
involved in degradation of glycoproteins
209051
Homo sapiens
2-acetamido-1-amino-1,2-dideoxy-beta-D-glucose + L-aspartate
-
209051
Homo sapiens
?
Other publictions for EC 3.5.1.26
No.
1st author
Pub Med
title
organims
journal
volume
pages
year
Activating Compound
Application
Cloned(Commentary)
Crystallization (Commentary)
Engineering
General Stability
Inhibitors
KM Value [mM]
Localization
Metals/Ions
Molecular Weight [Da]
Natural Substrates/ Products (Substrates)
Organic Solvent Stability
Organism
Oxidation Stability
Posttranslational Modification
Purification (Commentary)
Reaction
Renatured (Commentary)
Source Tissue
Specific Activity [micromol/min/mg]
Storage Stability
Substrates and Products (Substrate)
Subunits
Temperature Optimum [C]
Temperature Range [C]
Temperature Stability [C]
Turnover Number [1/s]
pH Optimum
pH Range
pH Stability
Cofactor
Ki Value [mM]
pI Value
IC50 Value
Activating Compound (protein specific)
Application (protein specific)
Cloned(Commentary) (protein specific)
Cofactor (protein specific)
Crystallization (Commentary) (protein specific)
Engineering (protein specific)
General Stability (protein specific)
IC50 Value (protein specific)
Inhibitors (protein specific)
Ki Value [mM] (protein specific)
KM Value [mM] (protein specific)
Localization (protein specific)
Metals/Ions (protein specific)
Molecular Weight [Da] (protein specific)
Natural Substrates/ Products (Substrates) (protein specific)
Organic Solvent Stability (protein specific)
Oxidation Stability (protein specific)
Posttranslational Modification (protein specific)
Purification (Commentary) (protein specific)
Renatured (Commentary) (protein specific)
Source Tissue (protein specific)
Specific Activity [micromol/min/mg] (protein specific)
Storage Stability (protein specific)
Substrates and Products (Substrate) (protein specific)
Subunits (protein specific)
Temperature Optimum [C] (protein specific)
Temperature Range [C] (protein specific)
Temperature Stability [C] (protein specific)
Turnover Number [1/s] (protein specific)
pH Optimum (protein specific)
pH Range (protein specific)
pH Stability (protein specific)
pI Value (protein specific)
Expression
General Information
General Information (protein specific)
Expression (protein specific)
KCat/KM [mM/s]
KCat/KM [mM/s] (protein specific)
735290
Sui
Structural basis of a point mu ...
Elizabethkingia miricola
Structure
22
1855-1861
2014
-
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1
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1
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2
2
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-
712162
Vinchon
Molecular and biochemical anal ...
Asobara tabida
Insect Biochem. Mol. Biol.
40
38-48
2010
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1
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7
1
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3
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1
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2
2
1
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1
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2
2
1
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1
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712651
Dunder
Early initiation of enzyme rep ...
Mus musculus
J. Inherit. Metab. Dis.
33
611-617
2010
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1
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1
1
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720247
Wang
Crystallographic snapshot of g ...
Flavobacterium sp.
J. Mol. Biol.
403
120-130
2010
-
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-
1
1
-
1
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1
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1
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1
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712919
Kelo
Depletion of L-asparagine supp ...
Homo sapiens
Leukemia
23
1167-1171
2009
-
1
-
-
-
-
-
1
1
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-
-
2
-
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1
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1
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1
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2
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681417
Wang
Crystallographic snapshot of a ...
Elizabethkingia meningoseptica
J. Mol. Biol.
366
82-92
2007
-
-
-
1
1
-
-
-
1
-
-
1
-
3
-
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-
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1
1
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2
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1
1
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1
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1
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-
1
1
-
-
-
2
-
-
-
-
-
-
-
-
-
-
682793
Mills
Diagnosis of congenital disord ...
Homo sapiens
Proteomics
6
2295-2304
2006
-
1
-
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-
-
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-
-
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-
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2
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1
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1
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-
1
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-
-
-
669745
Jackson
Elevation of plasma aspartylgl ...
Homo sapiens
J. Inherit. Metab. Dis.
28
1197-1198
2005
-
1
-
-
-
-
-
-
1
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1
-
2
-
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-
1
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1
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1
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1
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-
-
654537
Saarela
Autoproteolytic activation of ...
Homo sapiens
Biochem. J.
378
363-371
2004
1
1
1
-
11
-
-
3
2
-
1
-
-
3
-
1
1
1
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-
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2
2
1
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1
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1
1
1
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-
11
-
-
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3
2
-
1
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-
1
1
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-
2
2
1
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-
1
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-
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-
-
-
-
668879
Saarela
A novel aspartylglucosaminuria ...
Homo sapiens
Hum. Mutat.
24
350-351
2004
-
-
1
-
1
-
-
-
2
-
-
1
-
2
-
1
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1
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1
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1
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1
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-
-
-
-
-
-
-
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-
-
-
-
657367
Qian
A dual role for an aspartic ac ...
Elizabethkingia meningoseptica
Structure
11
997-1003
2003
1
-
-
1
1
-
1
-
-
-
-
-
-
1
-
1
1
-
-
-
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1
-
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1
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1
1
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1
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1
1
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-
-
1
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
654334
Risley
Glycosylasparaginase activity ...
Homo sapiens
Arch. Biochem. Biophys.
391
165-170
2001
1
1
-
-
-
-
-
-
-
-
-
1
-
1
-
1
-
-
-
-
-
-
3
-
-
-
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-
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-
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-
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1
1
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1
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1
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-
-
-
3
-
-
-
-
-
-
-
-
-
-
-
-
-
-
-
656376
Risley
Glycosylasparaginase inhibitio ...
Homo sapiens
J. Enzyme Inhib.
16
269-274
2001
1
1
-
-
-
-
11
1
-
-
-
1
-
2
-
1
-
1
-
1
-
-
2
1
1
-
-
-
1
-
-
-
10
-
-
1
1
-
-
-
-
-
-
11
10
1
-
-
-
1
-
-
1
-
-
1
-
-
2
1
1
-
-
-
1
-
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-
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-
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-
209049
Cui
Purification and crystallizati ...
Elizabethkingia meningoseptica
Acta Crystallogr. Sect. D
55
1961-1964
1999
-
-
1
1
1
1
-
-
-
-
-
1
-
3
-
-
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-
1
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1
1
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1
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1
1
1
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1
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1
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1
1
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-
209048
Saarela
Activation and oligomerization ...
Homo sapiens
J. Biol. Chem.
273
25320-25328
1998
-
-
1
-
3
-
-
-
1
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1
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1
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1
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1
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1
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1
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1
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1
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1
1
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-
209047
Noronkoski
Recombinant human glycosylaspa ...
Homo sapiens
FEBS Lett.
412
149-152
1997
-
-
1
-
-
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2
2
1
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2
-
2
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1
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1
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3
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1
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1
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2
1
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1
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2
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1
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1
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3
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1
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209050
Liu
Purification, biochemistry and ...
Bos taurus, Rattus sp., Spodoptera frugiperda
Glycobiology
6
527-536
1996
-
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1
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4
4
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8
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1
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3
1
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4
4
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1
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1
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1
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4
4
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1
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3
1
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4
4
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1
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1
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-
209051
Tikkanen
Functional analyses of active ...
Homo sapiens
EMBO J.
15
2954-2960
1996
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1
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6
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1
2
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1
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2
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1
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2
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1
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1
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6
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2
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1
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1
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2
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1
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209052
Park
Glycosylation and phosphorylat ...
Homo sapiens
Arch. Biochem. Biophys.
328
73-77
1996
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1
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3
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1
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3
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2
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1
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1
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209053
Mononen
Enzymatic diagnosis of asparty ...
Homo sapiens
Clin. Chem.
40
385-388
1994
-
1
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2
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6
2
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1
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1
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6
2
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1
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-
209054
Tarentino
The first demonstration of a p ...
Elizabethkingia meningoseptica
Biochem. Biophys. Res. Commun.
197
179-186
1993
-
-
-
-
-
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2
-
2
-
3
3
-
4
-
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1
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1
1
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4
1
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2
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2
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3
3
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1
-
1
1
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4
1
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-
-
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-
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-
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-
-
209055
Enomaa
-
Human aspartylglucosaminidase ...
Homo sapiens
Biochem. J.
286
613-618
1992
-
-
-
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2
-
1
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1
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1
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1
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1
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2
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1
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1
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1
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1
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-
-
-
-
209056
Rip
Purification and structure of ...
Homo sapiens
Biochem. J.
288
1005-1010
1992
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3
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2
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1
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2
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3
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1
1
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2
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1
1
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1
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209057
Tollersrud
Comparison of liver glycosylas ...
Bos taurus, Gallus gallus, Homo sapiens, Mus musculus, Rattus sp., Sus scrofa
Biochem. J.
282
891-897
1992
-
-
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-
6
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-
2
6
-
14
-
6
6
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12
6
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6
5
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6
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6
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6
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2
6
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6
6
-
12
6
-
6
5
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6
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6
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209058
Kaartinen
Glycoasparaginase in human uri ...
Homo sapiens
Biochim. Biophys. Acta
1097
28-30
1991
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1
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1
1
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3
1
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1
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2
1
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1
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1
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1
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1
1
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3
1
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1
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2
1
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1
1
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1
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1
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209059
Kaartinen
Glycosasparaginase from human ...
Homo sapiens
J. Biol. Chem.
266
5860-5869
1991
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-
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1
1
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3
1
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2
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1
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2
1
1
1
1
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1
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1
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1
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3
1
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1
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2
1
1
1
1
-
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1
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209060
Halila
Human leucocyte aspartylglucos ...
Homo sapiens
Biochem. J.
276
251-256
1991
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5
1
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2
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1
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2
1
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1
1
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5
1
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1
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2
1
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1
1
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209061
McGovern
Purification and properties of ...
Homo sapiens
J. Biol. Chem.
258
10743-10747
1983
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-
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1
2
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2
1
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2
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1
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2
1
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1
1
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1
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1
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1
2
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2
1
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1
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2
1
-
1
1
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-
1
-
1
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-
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209062
Dugal
Effect of different compounds ...
Homo sapiens
Biochem. J.
171
799-802
1978
3
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2
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1
1
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2
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1
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2
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1
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3
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2
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1
1
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1
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2
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1
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209063
Dugal
Purification and some properti ...
Homo sapiens
Biochem. J.
165
497-502
1977
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-
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5
1
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1
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2
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1
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2
2
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1
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1
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5
-
1
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1
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1
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2
2
-
1
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-
1
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-
-
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-
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209064
Dugal
Measurement of 1-aspartamido-b ...
Homo sapiens
Biochem. J.
163
9-14
1977
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-
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2
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1
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6
1
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1
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1
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6
1
-
1
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-
-
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-
209067
Tanaka
Specificity studies of 4-L-asp ...
Sus scrofa
J. Biochem.
73
1285-1289
1973
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-
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2
6
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1
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1
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2
1
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6
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2
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6
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1
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2
1
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6
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209068
Kohno
Purification and properties of ...
Sus scrofa
Biochim. Biophys. Acta
258
600-617
1972
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-
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1
6
3
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1
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1
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1
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1
1
1
3
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2
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1
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1
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1
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6
1
3
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1
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1
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1
1
1
3
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2
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1
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209065
Tarentino
The purification and propertie ...
Gallus gallus
Arch. Biochem. Biophys.
130
295-303
1969
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1
1
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1
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1
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1
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1
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1
4
-
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1
1
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1
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1
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1
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1
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1
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1
4
-
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1
1
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209066
Mahadevan
beta-Aspartylglucosylamine ami ...
Rattus sp.
J. Biol. Chem.
242
4568-4578
1967
-
-
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4
1
1
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1
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1
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2
1
1
1
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1
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1
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2
-
2
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4
2
1
1
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1
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2
1
1
1
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1
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1
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2
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