KEGG    Network variation - Sphingolipid degradation
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ENTRYnt06014
NameSphingolipid degradation
CategoryPathway view; Lipid/glycolipid metabolism
Pathwayhsa00600 Sphingolipid metabolism
DiseaseH00423 Sphingolipidosis
Display drug-target relation   disease type
N01710    GM1GLB1GM2(HEXA,HEXB)GM3NEU1LacCer
    GM1 gangliosidosis GM1 GLB1*
    Tay-Sachs disease     GM2 HEXA*
    Sandhoff disease     GM2 HEXB*
N01713        GM2A(HEXA,HEXB)
N01714    GM2 gangliosidosis, AB variant     GM2A*(HEXA,HEXB)
N01711    GA1GLB1GA2(HEXA,HEXB)LacCer
N01709        Gb4Cer(HEXA,HEXB)Gb3CerGLALacCer
           D4,D5,D6
 |
    Fabry disease         Gb3Cer GLA*
           D7
 |
    Fabry disease         Gb3Cer GLA*
 
N00640    LacCerGLB1GlcCerGBACeramide
       D1,D2,D3
 |
    Gaucher disease     GlcCer GBA*
   D9,D10
 |
   D1,D2,D3
 |
N00679    Gaucher disease CeramideUGCGGlcCerGBA*
N00642        PSAP(GBA,GALC)
N00643    Gaucher disease     PSAP*(GBA,GALC)
 
N00644    Ga2CerGLAGalCerGALCCeramide
N01712        PsychosineGALCSphingosine
    Krabbe disease     Psychosine GALC*
 
N00647    SulfatideARSAGalCer
    Metachromatic leukodystrophy   ARSA*
 
N00649    SphingomyelinSMPDCeramideASAHSphingosine
   D8
 |
    Niemann-Pick disease Sphingomyelin SMPD1*
    Farber lipogranulomatosis       ASAH1*

Disease nameDisease category
GM1 gangliosidosisH00281GM1 gangliosidosisInherited metabolic disorder, Lysosomal disease
Tay-Sachs diseaseH02016Tay-Sachs diseaseInherited metabolic disorder, Lysosomal disease
Sandhoff diseaseH02017Sandhoff diseaseInherited metabolic disorder, Lysosomal disease
GM2 gangliosidosis, AB variantH00124GM2 gangliosidosesInherited metabolic disorder, Lysosomal disease
Fabry diseaseH00125Fabry diseaseInherited metabolic disorder, Lysosomal disease
Gaucher diseaseH00126Gaucher diseaseInherited metabolic disorder, Lysosomal disease
Krabbe diseaseH00135Krabbe diseaseInherited metabolic disorder, Lysosomal disease
Metachromatic leukodystrophyH00127Metachromatic leukodystrophyInherited metabolic disorder, Lysosomal disease
Niemann-Pick diseaseH00137Niemann-Pick disease type A/BInherited metabolic disorder, Lysosomal disease
Farber lipogranulomatosisH00138Farber lipogranulomatosisInherited metabolic disorder, Lysosomal disease


Drug name
D1D03020Imiglucerase (USAN/INN)
D2D09029Velaglucerase alfa (USAN)
D3D09675Taliglucerase alfa (USAN/INN)
D4D02784Agalsidase alfa (USAN/INN)
D5D03228Agalsidase beta (INN)
D6D11685Pegunigalsidase alfa (USAN/INN)
D7D05031Migalastat hydrochloride (JAN/USAN)
D8D10820Olipudase alfa (USAN/INN)
D9D05032Miglustat (JAN/USAN/INN)
D10D09894Eliglustat tartrate (JAN/USAN)