General Information of Protein (ID: PRT01379)
Name Alpha-N-acetylglucosaminidase (NAGLU)
Gene Name Naglu Gene ID
27419
UniProt ID
O88325
Family Hydrolases (EC 3)
EC Number   EC: 3.2.1.50  (Click to Show/Hide the Complete EC Tree)
Hydrolases
Glycosylase
O/S-glycosyl compound glycosidase
EC: 3.2.1.50
  Click to Show/Hide the Molecular/Functional Data (Sequence/Structure/Function) of This Protein
Sequence
MEAAGLAVILGFLLLAGGSVGDEAREAKAVRELVVRLLGPGPAANFLVSVERALADESGL
DTYSLSGGGGVPVLVRGSTGVAAAAGLHRYLRDFCGCQVAWSSAQLHLPWPLPAVPDGLT
ETTPNRYRYYQNVCTHSYSFVWWDWARWEQEIDWMALNGINLALAWNGQEAIWQRVYLAL
GLTQSEIDTYFTGPAFLAWGRMGNLHTWDGPLPRSWHLSQVYLQHRILDRMRSFGMIPVL
PAFAGHVPKAITRVFPQVNVIKLGSWGHFNCSYSCSFLLAPGDPMFPLIGNLFLRELTKE
FGTDHIYGADTFNEMQPPFSDPSYLAATTAAVYEAMVTVDPDAVWLLQGWLFQHQPQFWG
PSQIRAVLEAVPRGRLLVLDLFAESHPVYMHTASFHGQPFIWCMLHNFGGNHGLFGALED
VNRGPQAARLFPNSTMVGTGIAPEGIGQNEVVYALMAELGWRKDPVPDLMAWVSSFAIRR
YGVSQPDAVAAWKLLLRSVYNCSGEACSGHNRSPLVKRPSLQMSTAVWYNRSDVFEAWRL
LLTAAPNLTTSPAFRYDLLDVTRQAVQELVSLCYEEARTAYLKQELDLLLRAGGLLVYKL
LPTLDELLASSSHFLLGTWLDQARKAAVSEAEAQFYEQNSRYQITLWGPEGNILDYANKQ
LAGLVADYYQPRWCLFLGTLAHSLARGVPFQQHEFEKNVFPLEQAFVYNKKRYPSQPRGD
TVDLSKKIFLKYHPQPDSL
Regulatory Network
Full List of Metabolite(s) Regulated by This Protein
      Lipids and lipid-like molecules
            4-Hydroxybutyric acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change 4-Hydroxybutyric acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of 4-hydroxybutyric acid levels compared with control group.
      Organic acids and derivatives
            Alanine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Alanine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of alanine levels compared with control group.
            Asparagine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Asparagine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of asparagine levels compared with control group.
            Aspartic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Aspartic acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of aspartic acid levels compared with control group.
            Cysteine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Cysteine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of cysteine levels compared with control group.
            Glutamic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Glutamic acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of glutamic acid levels compared with control group.
            Glutamine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Glutamine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of glutamine levels compared with control group.
            Glycine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Glycine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of glycine levels compared with control group.
            Histidine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Histidine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of histidine levels compared with control group.
            Isoleucine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Isoleucine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of isoleucine levels compared with control group.
            Leucine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Leucine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of leucine levels compared with control group.
            Lysine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Lysine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of lysine levels compared with control group.
            Methionine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Methionine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of methionine levels compared with control group.
            N-Acetyl-L-tyrosine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change N-Acetyl-L-tyrosine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of N-acetyl-L-tyrosine levels compared with control group.
            Phenylalanine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Phenylalanine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of phenylalanine levels compared with control group.
            Proline Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Proline concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of proline levels compared with control group.
            Valine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Valine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of valine levels compared with control group.
      Organic oxygen compounds
            D-Mannose Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change D-Mannose concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of D-Mannose levels compared with control group.
            Erythronic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Erythronic acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of erythronic acid levels compared with control group.
            Fructose Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Fructose concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of fructose levels compared with control group.
            Fucose Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Fucose concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of fucose levels compared with control group.
            Glucose Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Glucose concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of glucose levels compared with control group.
            N-Acetyl-D-glucosamine Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change N-Acetyl-D-glucosamine concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of N-acetyl-D-glucosamine levels compared with control group.
            N-Acetylneuraminic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change N-Acetylneuraminic acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of N-acetylneuraminic acid levels compared with control group.
      Organoheterocyclic compounds
            5-Hydroxyindoleacetic acid Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change 5-Hydroxyindoleacetic acid concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of 5-hydroxyindoleacetic acid levels compared with control group.
            Dihydrobiopterin Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Dihydrobiopterin concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of dihydrobiopterin levels compared with control group.
            Serotonin Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Serotonin concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of serotonin levels compared with control group.
            Tryptophan Click to Show/Hide the Full List of Regulating Pair(s):   1 Pair(s)
               Detailed Information Metabo  Info click to show the details of this metabolite
               Regulating Pair Experim Info click to show the details of experiment for validating this pair [1]
                      Introduced Variation Knockout of Naglu
                      Induced Change Tryptophan concentration: decrease
                      Summary Introduced Variation         Induced Change 
                      Disease Status Lysosomal storage diseases [ICD-11: 5C56]
                      Details It is reported that knockout of Naglu leads to the decrease of tryptophan levels compared with control group.
References
1 Near-Complete Correction of Profound Metabolomic Impairments Corresponding to Functional Benefit in MPS IIIB Mice after IV rAAV9-hNAGLU Gene Delivery. Mol Ther. 2017 Mar 1;25(3):792-802.

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