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ENTRY	nt06014
Name	Sphingolipid degradation
Category	Pathway view; Lipid/glycolipid metabolism
Pathway	hsa00600 Sphingolipid metabolism
Disease	H00423 Sphingolipidosis
Display	drug-target relation   disease type

N01710    GM1 − GLB1 → GM2 − (HEXA,HEXB) → GM3 − NEU1 → LacCer     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    GA1 − GLB1 → GA2 − (HEXA,HEXB) → LacCer N01709            Gb4Cer − (HEXA,HEXB) → Gb3Cer − GLA → LacCer                       D4,D5,D6  |     Fabry disease                 Gb3Cer↗   GLA*                       D7  |     Fabry disease                 Gb3Cer↗   GLA* N00640    LacCer − GLB1 → GlcCer − GBA → Ceramide               D1,D2,D3  |     Gaucher disease         GlcCer↗   GBA*       D9  |       D1,D2,D3  | N00679    Gaucher disease Ceramide − UGCG → GlcCer ⌿ GBA* N00642            PSAP → (GBA,GALC) N00643    Gaucher disease         PSAP* ⌿ (GBA,GALC) N00644    Ga2Cer − GLA → GalCer − GALC → Ceramide N01712            Psychosine − GALC → Sphingosine     Krabbe disease         Psychosine↗   GALC* N00647    Sulfatide − ARSA → GalCer     Metachromatic leukodystrophy     ARSA* N00649    Sphingomyelin − SMPD → Ceramide − ASAH → Sphingosine       D8  |     Niemann-Pick disease Sphingomyelin↗   SMPD1*     Farber lipogranulomatosis             ASAH1*

		Disease name	Disease category
GM1 gangliosidosis	H00281	GM1 gangliosidosis	Inherited metabolic disorder, Lysosomal disease
Tay-Sachs disease	H02016	Tay-Sachs disease	Inherited metabolic disorder, Lysosomal disease
Sandhoff disease	H02017	Sandhoff disease	Inherited metabolic disorder, Lysosomal disease
GM2 gangliosidosis, AB variant	H00124	GM2 gangliosidoses	Inherited metabolic disorder, Lysosomal disease
Fabry disease	H00125	Fabry disease	Inherited metabolic disorder, Lysosomal disease
Gaucher disease	H00126	Gaucher disease	Inherited metabolic disorder, Lysosomal disease
Krabbe disease	H00135	Krabbe disease	Inherited metabolic disorder, Lysosomal disease
Metachromatic leukodystrophy	H00127	Metachromatic leukodystrophy	Inherited metabolic disorder, Lysosomal disease
Niemann-Pick disease	H00137	Niemann-Pick disease type A/B	Inherited metabolic disorder, Lysosomal disease
Farber lipogranulomatosis	H00138	Farber lipogranulomatosis	Inherited metabolic disorder, Lysosomal disease

		Drug name
D1	D03020	Imiglucerase (USAN/INN)
D2	D09029	Velaglucerase alfa (USAN)
D3	D09675	Taliglucerase alfa (USAN/INN)
D4	D02784	Agalsidase alfa (USAN/INN)
D5	D03228	Agalsidase beta (INN)
D6	D11685	Pegunigalsidase alfa (USAN/INN)
D7	D05031	Migalastat hydrochloride (JAN/USAN)
D8	D10820	Olipudase alfa (USAN/INN)
D9	D09894	Eliglustat tartrate (JAN/USAN)