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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
GUSB
glucuronidase beta
Chromosome 7 Β· 7q11.21
NCBI Gene: 2990Ensembl: ENSG00000169919.18HGNC: HGNC:4696UniProt: P08236
94PubMed Papers
21Diseases
0Drugs
107Pathogenic Variants
RESEARCH IMPACT
Variant-Rich
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
GO:0005615hyaluronan catabolic processbeta-glucuronidase activitysignaling receptor bindingmucopolysaccharidosis type 7mucopolysaccharidosis type 6genetic disorderNon-immune hydrops fetalis
✦AI Summary

GUSB encodes glucuronidase beta, a lysosomal enzyme essential for degrading glycosaminoglycans (GAGs) including heparan sulfate, dermatan sulfate, and chondroitin sulfate 1. The enzyme plays a primary role in GAG catabolism, which is critical for normal connective tissue homeostasis 1. Deficiency of GUSB causes mucopolysaccharidosis VII (Sly syndrome), an autosomal recessive lysosomal storage disorder characterized by GAG accumulation in lysosomes, leading to mental retardation, short stature, hepatosplenomegaly, bone dysplasia, and hydrops fetalis 1. Over 49 unique disease-causing mutations have been identified in the GUSB gene, with significant genotype-phenotype correlation 1. Recently, elevated GUSB expression derived from dysbiotic gut microbiota has been implicated in endometriosis pathogenesis, promoting endometrial stromal cell proliferation and M2 macrophage transition 2. Emerging evidence suggests GUSB haploinsufficiency may represent a genetic risk factor for Alzheimer's disease; heterozygous loss-of-function mutations in GUSB increase amyloid-beta plaque formation in transgenic mouse models 3. GUSB is included among 25 genes recommended by 85% or more of rare disease experts for newborn screening via genome sequencing 4, reflecting its clinical significance in treatable metabolic disorders.

Sources cited
1
GUSB encodes beta-glucuronidase required to degrade glycosaminoglycans; GUSB deficiency causes mucopolysaccharidosis VII with characteristic clinical features and extensive genetic heterogeneity
PMID: 19224584
2
Dysbiosis-derived GUSB promotes endometriosis development by enhancing endometrial stromal cell proliferation and promoting M2 macrophage differentiation
PMID: 37178109
3
Heterozygous GUSB mutations are enriched in Alzheimer's disease patients and increase amyloid-beta plaque formation in mouse models
PMID: 39605615
4
GUSB was recommended by β‰₯85% of rare disease experts for inclusion in newborn genome sequencing for treatable conditions
PMID: 37155167
⚠Limited data available β€” This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsβ“˜21
mucopolysaccharidosis type 7Open Targets
0.88Strong
mucopolysaccharidosis type 6Open Targets
0.43Moderate
genetic disorderOpen Targets
0.41Moderate
Non-immune hydrops fetalisOpen Targets
0.39Weak
mucopolysaccharidosisOpen Targets
0.37Weak
hydrops fetalisOpen Targets
0.37Weak
Alzheimer diseaseOpen Targets
0.26Weak
gastritisOpen Targets
0.17Weak
neurodegenerative diseaseOpen Targets
0.13Weak
prostate cancerOpen Targets
0.11Weak
Familial prostate cancerOpen Targets
0.11Weak
neoplasmOpen Targets
0.10Weak
hepatocellular carcinomaOpen Targets
0.08Suggestive
head and neck squamous cell carcinomaOpen Targets
0.06Suggestive
pneumocystosisOpen Targets
0.06Suggestive
Pyle diseaseOpen Targets
0.06Suggestive
infectionOpen Targets
0.06Suggestive
multiple epiphyseal dysplasia type 1Open Targets
0.05Suggestive
metaphyseal dysplasia without hypotrichosisOpen Targets
0.05Suggestive
Schmid metaphyseal chondrodysplasiaOpen Targets
0.05Suggestive
Mucopolysaccharidosis 7UniProt
Pathogenic Variants107
NM_000181.4(GUSB):c.738C>G (p.Tyr246Ter)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2026β†’ Residue 246
NM_000181.4(GUSB):c.499C>T (p.Arg167Ter)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2026β†’ Residue 167
NM_000181.4(GUSB):c.526C>T (p.Leu176Phe)Pathogenic
Mucopolysaccharidosis type 7|Mucopolysaccharidosis type 6|not provided|GUSB-related disorder
β˜…β˜…β˜†β˜†2026β†’ Residue 176
NM_000181.4(GUSB):c.1069C>T (p.Arg357Ter)Pathogenic
Mucopolysaccharidosis type 7|Non-immune hydrops fetalis|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 357
NM_000181.4(GUSB):c.1856C>T (p.Ala619Val)Pathogenic
Mucopolysaccharidosis type 7|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 619
NM_000181.4(GUSB):c.1337G>A (p.Trp446Ter)Pathogenic
Mucopolysaccharidosis type 6|Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2025β†’ Residue 446
NM_000181.4(GUSB):c.1617C>T (p.Ser539=)Pathogenic
Mucopolysaccharidosis type 7|Uterine corpus endometrial carcinoma
β˜…β˜…β˜†β˜†2025β†’ Residue 539
NM_000181.4(GUSB):c.507dup (p.Ile170fs)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2025β†’ Residue 170
NM_000181.4(GUSB):c.1144C>T (p.Arg382Cys)Pathogenic
Mucopolysaccharidosis type 7|Non-immune hydrops fetalis
β˜…β˜…β˜†β˜†2025β†’ Residue 382
NM_000181.4(GUSB):c.1881G>T (p.Trp627Cys)Pathogenic
Mucopolysaccharidosis type 7|not provided|GUSB-related disorder
β˜…β˜…β˜†β˜†2025β†’ Residue 627
NM_000181.4(GUSB):c.1623T>A (p.Tyr541Ter)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2025β†’ Residue 541
NM_000181.4(GUSB):c.1521G>A (p.Trp507Ter)Pathogenic
Mucopolysaccharidosis type 7|not provided
β˜…β˜…β˜†β˜†2025β†’ Residue 507
NM_000181.4(GUSB):c.163C>T (p.Arg55Ter)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2025β†’ Residue 55
NM_000181.4(GUSB):c.724+1G>TPathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2024
NM_000181.4(GUSB):c.1455dup (p.Asn486Ter)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2024β†’ Residue 486
NM_000181.4(GUSB):c.91C>T (p.Gln31Ter)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2024β†’ Residue 31
NM_000181.4(GUSB):c.328C>T (p.Arg110Ter)Pathogenic
not provided|Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2024β†’ Residue 110
NM_000181.4(GUSB):c.1874_1875del (p.Arg625fs)Pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2024β†’ Residue 625
NM_000181.4(GUSB):c.646C>T (p.Arg216Trp)Pathogenic
Mucopolysaccharidosis type 7|not provided
β˜…β˜…β˜†β˜†2023β†’ Residue 216
NM_000181.4(GUSB):c.1476_1476+13delLikely pathogenic
Mucopolysaccharidosis type 7
β˜…β˜…β˜†β˜†2023
View on ClinVar β†—
Related Genes
AKR1A1Protein interaction97%UGT1A9Protein interaction90%UGT2B10Protein interaction90%UGT2B7Protein interaction90%UGT2A2Protein interaction90%UGT1A5Protein interaction90%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
62%
Lung
36%
Ovary
29%
Brain
18%
Heart
18%
Gene Interaction Network
Click a node to explore
GUSBAKR1A1UGT1A9UGT2B10UGT2B7UGT2A2UGT1A5
PROTEIN STRUCTURE
Preparing viewer…
PDB3HN3 Β· 1.70 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.87LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.68 [0.54–0.87]
RankingsWhere GUSB stands among ~20K protein-coding genes
  • #5,084of 20,598
    Most Researched94 Β· top quartile
  • #726of 5,498
    Most Pathogenic Variants107 Β· top quartile
  • #7,680of 17,882
    Most Constrained (LOEUF)0.87
Genes detectedGUSB
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Novel vectors and approaches for gene therapy in liver diseases.
PMID: 34159305
JHEP Rep Β· 2021
1.00
2
Gut dysbiosis-derived Ξ²-glucuronidase promotes the development of endometriosis.
PMID: 37178109
Fertil Steril Β· 2023
0.90
3
Metabolic Cardiomyopathies and Cardiac Defects in Inherited Disorders of Carbohydrate Metabolism: A Systematic Review.
PMID: 37239976
Int J Mol Sci Β· 2023
0.80
4
Mutations and polymorphisms in GUSB gene in mucopolysaccharidosis VII (Sly Syndrome).
PMID: 19224584
Hum Mutat Β· 2009
0.70
5
AMPK protects proximal tubular epithelial cells from lysosomal dysfunction and dedifferentiation induced by lipotoxicity.
PMID: 39675352
Autophagy Β· 2025
0.60