KEGG   PATHWAY: hsa00531Help
Entry
hsa00531                    Pathway                                

Name
Glycosaminoglycan degradation - Homo sapiens (human)
Class
Metabolism; Glycan biosynthesis and metabolism
BRITE hierarchy
Pathway map
Glycosaminoglycan degradation
hsa00531

All organismsOrtholog table
Module
Dermatan sulfate degradation [PATH:hsa00531]
Chondroitin sulfate degradation [PATH:hsa00531]
Heparan sulfate degradation [PATH:hsa00531]
Keratan sulfate degradation [PATH:hsa00531]
Disease
H00123  
Mucopolysaccharidosis type IV (MPS4)
H00124  
GM2 gangliosidoses
H00128  
Mucopolysaccharidosis type I (MPS1)
H00129  
Mucopolysaccharidosis type II (MPS2)
H00130  
Mucopolysaccharidosis type III (MPS3)
H00131  
Mucopolysaccharidosis type VI (MPS6)
H00132  
Mucopolysaccharidosis type VII (MPS7)
H00133  
Mucopolysaccharidosis type IX (MPS9)
H00281  
GM1 gangliosidosis
H00421  
Mucopolysaccharidosis (MPS)
H00426  
Defects in the degradation of ganglioside
H00753  
Urofacial syndrome
Organism
Homo sapiens (human) [GN:hsa]
Gene
8692  
HYAL2; hyaluronoglucosaminidase 2 [KO:K01197] [EC:3.2.1.35]
3373  
HYAL1; hyaluronoglucosaminidase 1 [KO:K01197] [EC:3.2.1.35]
6677  
SPAM1; sperm adhesion molecule 1 [KO:K01197] [EC:3.2.1.35]
23553  
HYAL4; hyaluronoglucosaminidase 4 [KO:K01197] [EC:3.2.1.35]
8372  
HYAL3; hyaluronoglucosaminidase 3 [KO:K01197] [EC:3.2.1.35]
2990  
GUSB; glucuronidase beta [KO:K01195] [EC:3.2.1.31]
3423  
IDS; iduronate 2-sulfatase [KO:K01136] [EC:3.1.6.13]
3425  
IDUA; iduronidase, alpha-L- [KO:K01217] [EC:3.2.1.76]
411  
ARSB; arylsulfatase B [KO:K01135] [EC:3.1.6.12]
10855  
HPSE; heparanase [KO:K07964] [EC:3.2.1.166]
60495  
HPSE2; heparanase 2 (inactive) [KO:K07965] [EC:3.2.1.-]
6448  
SGSH; N-sulfoglucosamine sulfohydrolase [KO:K01565] [EC:3.10.1.1]
138050  
HGSNAT; heparan-alpha-glucosaminide N-acetyltransferase [KO:K10532] [EC:2.3.1.78]
4669  
NAGLU; N-acetylglucosaminidase, alpha [KO:K01205] [EC:3.2.1.50]
2588  
GALNS; galactosamine (N-acetyl)-6-sulfatase [KO:K01132] [EC:3.1.6.4]
2720  
GLB1; galactosidase beta 1 [KO:K12309] [EC:3.2.1.23]
2799  
GNS; glucosamine (N-acetyl)-6-sulfatase [KO:K01137] [EC:3.1.6.14]
3073  
HEXA; hexosaminidase subunit alpha [KO:K12373] [EC:3.2.1.52]
3074  
HEXB; hexosaminidase subunit beta [KO:K12373] [EC:3.2.1.52]
Compound
C00426  
Dermatan sulfate
C00573  
Keratan sulfate
C00634  
Chondroitin 4-sulfate
C00925  
Heparan sulfate
G00526  
 
G00711  
 
G00872  
DWA-2
G01391  
 
G01945  
 
G01977  
 
G02632  
 
G09660  
 
G10008  
 
G10505  
Hyaluronic acid
G12336  
 
G13034  
 
G13035  
 
G13036  
 
G13037  
 
G13038  
 
G13039  
 
G13040  
 
G13041  
 
G13042  
 
G13043  
 
G13073  
 
G13074  
 
Reference
PMID:27570
  Authors
Greiling H, Stuhlsatz HW, Cantz M, Gehler J
  Title
Increased urinary excretion of keratan sulfate in fucosidosis.
  Journal
J Clin Chem Clin Biochem 16:329-34 (1978)
Reference
PMID:3161730
  Authors
Fuchs W, Beck M, Kresse H
  Title
Intralysosomal formation and metabolic fate of N-acetylglucosamine 6-sulfate from keratan sulfate.
  Journal
Eur J Biochem 151:551-6 (1985)
KO pathway
 

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