Gene/Proteome Database (LMPD)
LMPD ID
LMP005477
Gene ID
Species
Homo sapiens (Human)
Gene Name
glucuronidase, beta
Gene Symbol
Synonyms
BG; MPS7
Chromosome
7
Map Location
7q21.11
EC Number
3.2.1.31
Summary
This gene encodes a hydrolase that degrades glycosaminoglycans, including heparan sulfate, dermatan sulfate, and chondroitin-4,6-sulfate. The enzyme forms a homotetramer that is localized to the lysosome. Mutations in this gene result in mucopolysaccharidosis type VII. Alternative splicing results in multiple transcript variants. There are many pseudogenes of this locus in the human genome.[provided by RefSeq, May 2014]
Orthologs
Proteins
| beta-glucuronidase isoform 1 precursor | |
|---|---|
| Refseq ID | NP_000172 |
| Protein GI | 268834192 |
| UniProt ID | P08236 |
| mRNA ID | NM_000181 |
| Length | 651 |
| RefSeq Status | REVIEWED |
| MARGSAVAWAALGPLLWGCALGLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWESGPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVLRIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAINNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYTTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVANGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYTLPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFNLLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNVSLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKSLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHLGLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFLLRERYWKIANETRYPHSVAKSQCLENSLFT | |
| beta-glucuronidase isoform 2 precursor | |
|---|---|
| Refseq ID | NP_001271219 |
| Protein GI | 268834192 |
| UniProt ID | P08236 |
| mRNA ID | NM_001284290 |
| Length | 651 |
| RefSeq Status | REVIEWED |
| MARGSAVAWAALGPLLWGCALGLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWESGPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVLRIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAINNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYTTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVANGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYTLPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFNLLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNVSLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKSLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHLGLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFLLRERYWKIANETRYPHSVAKSQCLENSLFT | |
| beta-glucuronidase isoform 3 | |
|---|---|
| Refseq ID | NP_001280033 |
| Protein GI | 268834192 |
| UniProt ID | P08236 |
| mRNA ID | NM_001293104 |
| Length | 651 |
| RefSeq Status | REVIEWED |
| MARGSAVAWAALGPLLWGCALGLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWESGPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVLRIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAINNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYTTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVANGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYTLPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFNLLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNVSLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKSLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHLGLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFLLRERYWKIANETRYPHSVAKSQCLENSLFT | |
| beta-glucuronidase isoform 4 | |
|---|---|
| Refseq ID | NP_001280034 |
| Protein GI | 268834192 |
| UniProt ID | P08236 |
| mRNA ID | NM_001293105 |
| Length | 651 |
| RefSeq Status | REVIEWED |
| MARGSAVAWAALGPLLWGCALGLQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWESGPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVLRIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAINNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYTTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVANGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYTLPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFNLLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNVSLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKSLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHLGLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFLLRERYWKIANETRYPHSVAKSQCLENSLFT | |
| sig_peptide: 1..22 inference: COORDINATES: ab initio prediction:SignalP:4.0 calculated_mol_wt: 2172 peptide sequence: MARGSAVAWAALGPLLWGCALG mat_peptide: 23..651 product: beta-glucuronidase isoform 1 calculated_mol_wt: 72579 peptide sequence: LQGGMLYPQESPSRECKELDGLWSFRADFSDNRRRGFEEQWYRRPLWESGPTVDMPVPSSFNDISQDWRLRHFVGWVWYEREVILPERWTQDLRTRVVLRIGSAHSYAIVWVNGVDTLEHEGGYLPFEADISNLVQVGPLPSRLRITIAINNTLTPTTLPPGTIQYLTDTSKYPKGYFVQNTYFDFFNYAGLQRSVLLYTTPTTYIDDITVTTSVEQDSGLVNYQISVKGSNLFKLEVRLLDAENKVVANGTGTQGQLKVPGVSLWWPYLMHERPAYLYSLEVQLTAQTSLGPVSDFYTLPVGIRTVAVTKSQFLINGKPFYFHGVNKHEDADIRGKGFDWPLLVKDFNLLRWLGANAFRTSHYPYAEEVMQMCDRYGIVVIDECPGVGLALPQFFNNVSLHHHMQVMEEVVRRDKNHPAVVMWSVANEPASHLESAGYYLKMVIAHTKSLDPSRPVTFVSNSNYAADKGAPYVDVICLNSYYSWYHDYGHLELIQLQLATQFENWYKKYQKPIIQSEYGAETIAGFHQDPPLMFTEEYQKSLLEQYHLGLDQKRRKYVVGELIWNFADFMTEQSPTRVLGNKKGIFTRQRQPKSAAFLLRERYWKIANETRYPHSVAKSQCLENSLFT sig_peptide: 1..22 inference: COORDINATES: ab initio prediction:SignalP:4.0 calculated_mol_wt: 2172 peptide sequence: MARGSAVAWAALGPLLWGCALG | |
Gene Information
Gene Ontology (GO Annotations)
| GO ID | Source | Type | Description |
|---|---|---|---|
| GO:0070062 | IDA:UniProtKB | C | extracellular vesicular exosome |
| GO:0043231 | IDA:HPA | C | intracellular membrane-bounded organelle |
| GO:0043202 | TAS:Reactome | C | lysosomal lumen |
| GO:0016020 | IDA:UniProtKB | C | membrane |
| GO:0004566 | TAS:Reactome | F | beta-glucuronidase activity |
| GO:0005975 | TAS:Reactome | P | carbohydrate metabolic process |
| GO:0006027 | TAS:Reactome | P | glycosaminoglycan catabolic process |
| GO:0030203 | TAS:Reactome | P | glycosaminoglycan metabolic process |
| GO:0030214 | TAS:Reactome | P | hyaluronan catabolic process |
| GO:0030212 | TAS:Reactome | P | hyaluronan metabolic process |
| GO:0044281 | TAS:Reactome | P | small molecule metabolic process |
KEGG Pathway Links
| KEGG Pathway ID | Description |
|---|---|
| hsa04142 | Lysosome |
REACTOME Pathway Links
| REACTOME Pathway ID | Description |
|---|---|
| REACT_120752 | HS-GAG degradation |
| REACT_120996 | Hyaluronan uptake and degradation |
Domain Information
InterPro Annotations
| Accession | Description |
|---|---|
| IPR008979 | Galactose-binding domain-like |
| IPR017853 | Glycoside hydrolase superfamily |
| IPR013781 | Glycoside hydrolase, catalytic domain |
| IPR006101 | Glycoside hydrolase, family 2 |
| IPR006103 | Glycoside hydrolase, family 2, TIM barrel |
| IPR023232 | Glycoside hydrolase, family 2, active site |
| IPR023230 | Glycoside hydrolase, family 2, conserved site |
| IPR006102 | Glycoside hydrolase, family 2, immunoglobulin-like beta-sandwich |
| IPR013812 | Glycoside hydrolase, family 2/20, immunoglobulin-like beta-sandwich domain |
| IPR006104 | Glycosyl hydrolases family 2, sugar binding domain |
UniProt Annotations
Entry Information
Comments
| Comment Type | Description |
|---|---|
| Alternative Products | Event=Alternative splicing; Named isoforms=3; Name=1; Synonyms=Long; IsoId=P08236-1; Sequence=Displayed; Name=2; Synonyms=Short; IsoId=P08236-2; Sequence=VSP_001799; Name=3; IsoId=P08236-3; Sequence=VSP_054830; Note=No experimental confirmation available.; |
| Catalytic Activity | A beta-D-glucuronoside + H(2)O = D-glucuronate + an alcohol. |
| Disease | Mucopolysaccharidosis 7 (MPS7) [MIM |
| Disease | Note=Mucopolysaccharidosis type 7 is associated with non- immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. |
| Enzyme Regulation | Inhibited by L-aspartic acid. |
| Function | Plays an important role in the degradation of dermatan and keratan sulfates. |
| Ptm | N-linked glycosylated with 3 to 4 oligosaccharide chains. {ECO |
| Similarity | Belongs to the glycosyl hydrolase 2 family. |
| Subcellular Location | Lysosome. |
| Subunit | Homotetramer. |
Identical and Related Proteins
Unique RefSeq proteins for LMP005477 (as displayed in Record Overview)
| Protein GI | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| 268834192 | RefSeq | NP_000172 | 651 | beta-glucuronidase isoform 1 precursor |
| 268834192 | RefSeq | NP_001271219 | 651 | beta-glucuronidase isoform 2 precursor |
| 268834192 | RefSeq | NP_001280033 | 651 | beta-glucuronidase isoform 3 |
| 268834192 | RefSeq | NP_001280034 | 651 | beta-glucuronidase isoform 4 |
Identical Sequences to LMP005477 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:268834192 | DBBJ | BAD97126.1 | 651 | glucuronidase, beta variant, partial [Homo sapiens] |
| GI:268834192 | DBBJ | BAD97126.1 | 651 | glucuronidase, beta variant, partial [Homo sapiens] |
| GI:268834192 | DBBJ | BAD97126.1 | 651 | glucuronidase, beta variant, partial [Homo sapiens] |
| GI:268834192 | DBBJ | BAD97126.1 | 651 | glucuronidase, beta variant, partial [Homo sapiens] |
| GI:268834192 | GenBank | EAX07951.1 | 651 | glucuronidase, beta, isoform CRA_c [Homo sapiens] |
| GI:268834192 | GenBank | EAX07951.1 | 651 | glucuronidase, beta, isoform CRA_c [Homo sapiens] |
| GI:268834192 | GenBank | EAX07951.1 | 651 | glucuronidase, beta, isoform CRA_c [Homo sapiens] |
| GI:268834192 | GenBank | EAX07951.1 | 651 | glucuronidase, beta, isoform CRA_c [Homo sapiens] |
| GI:268834192 | GenBank | ABM83867.1 | 651 | glucuronidase, beta [synthetic construct] |
| GI:268834192 | GenBank | ABM83867.1 | 651 | glucuronidase, beta [synthetic construct] |
| GI:268834192 | GenBank | ABM83867.1 | 651 | glucuronidase, beta [synthetic construct] |
| GI:268834192 | GenBank | ABM83867.1 | 651 | glucuronidase, beta [synthetic construct] |
| GI:268834192 | GenBank | ABM87189.1 | 651 | glucuronidase, beta, partial [synthetic construct] |
| GI:268834192 | GenBank | ABM87189.1 | 651 | glucuronidase, beta, partial [synthetic construct] |
| GI:268834192 | GenBank | ABM87189.1 | 651 | glucuronidase, beta, partial [synthetic construct] |
| GI:268834192 | GenBank | ABM87189.1 | 651 | glucuronidase, beta, partial [synthetic construct] |
| GI:268834192 | GenBank | AIC48903.1 | 651 | GUSB, partial [synthetic construct] |
| GI:268834192 | GenBank | AIC48903.1 | 651 | GUSB, partial [synthetic construct] |
| GI:268834192 | GenBank | AIC48903.1 | 651 | GUSB, partial [synthetic construct] |
| GI:268834192 | GenBank | AIC48903.1 | 651 | GUSB, partial [synthetic construct] |
| GI:268834192 | SwissProt | P08236.2 | 651 | RecName: Full=Beta-glucuronidase; AltName: Full=Beta-G1; Flags: Precursor [Homo sapiens] |
| GI:268834192 | SwissProt | P08236.2 | 651 | RecName: Full=Beta-glucuronidase; AltName: Full=Beta-G1; Flags: Precursor [Homo sapiens] |
| GI:268834192 | SwissProt | P08236.2 | 651 | RecName: Full=Beta-glucuronidase; AltName: Full=Beta-G1; Flags: Precursor [Homo sapiens] |
| GI:268834192 | SwissProt | P08236.2 | 651 | RecName: Full=Beta-glucuronidase; AltName: Full=Beta-G1; Flags: Precursor [Homo sapiens] |
Related Sequences to LMP005477 proteins
| Reference | Database | Accession | Length | Protein Name |
|---|---|---|---|---|
| GI:268834192 | GenBank | ABU31314.1 | 651 | Sequence 9336 from patent US 7235358 |
| GI:268834192 | GenBank | ABU31314.1 | 651 | Sequence 9336 from patent US 7235358 |
| GI:268834192 | GenBank | ABU31314.1 | 651 | Sequence 9336 from patent US 7235358 |
| GI:268834192 | GenBank | ABU31314.1 | 651 | Sequence 9336 from patent US 7235358 |
| GI:268834192 | GenBank | ADS18551.1 | 651 | Sequence 41 from patent US 7763438 |
| GI:268834192 | GenBank | ADS18551.1 | 651 | Sequence 41 from patent US 7763438 |
| GI:268834192 | GenBank | ADS18551.1 | 651 | Sequence 41 from patent US 7763438 |
| GI:268834192 | GenBank | ADS18551.1 | 651 | Sequence 41 from patent US 7763438 |
| GI:268834192 | GenBank | AED17283.1 | 651 | Sequence 2401 from patent US 7879556 |
| GI:268834192 | GenBank | AED17283.1 | 651 | Sequence 2401 from patent US 7879556 |
| GI:268834192 | GenBank | AED17283.1 | 651 | Sequence 2401 from patent US 7879556 |
| GI:268834192 | GenBank | AED17283.1 | 651 | Sequence 2401 from patent US 7879556 |
| GI:268834192 | GenBank | AED17628.1 | 651 | Sequence 12453 from patent US 7879556 |
| GI:268834192 | GenBank | AED17628.1 | 651 | Sequence 12453 from patent US 7879556 |
| GI:268834192 | GenBank | AED17628.1 | 651 | Sequence 12453 from patent US 7879556 |
| GI:268834192 | GenBank | AED17628.1 | 651 | Sequence 12453 from patent US 7879556 |
| GI:268834192 | GenBank | AED47610.1 | 651 | Sequence 2401 from patent US 7892745 |
| GI:268834192 | GenBank | AED47610.1 | 651 | Sequence 2401 from patent US 7892745 |
| GI:268834192 | GenBank | AED47610.1 | 651 | Sequence 2401 from patent US 7892745 |
| GI:268834192 | GenBank | AED47610.1 | 651 | Sequence 2401 from patent US 7892745 |
| GI:268834192 | GenBank | AED47955.1 | 651 | Sequence 12453 from patent US 7892745 |
| GI:268834192 | GenBank | AED47955.1 | 651 | Sequence 12453 from patent US 7892745 |
| GI:268834192 | GenBank | AED47955.1 | 651 | Sequence 12453 from patent US 7892745 |
| GI:268834192 | GenBank | AED47955.1 | 651 | Sequence 12453 from patent US 7892745 |