KEGG    Network variation - Lysosome
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ENTRYnt06551
NameLysosome
CategoryPathway view; Cellular process
Pathwayhsa04142 Lysosome biogenesis
DiseaseH01425 Lysosomal storage disease
Display drug-target relation   disease type
N01710    GM1−GLB1→GM2−(HEXA,HEXB)→GM3−NEU1→LacCer
    GM1G   GLB1*
    TSD       HEXA*
    SD       HEXB*
    Sialidosis           NEU1*
N01713        GM2A→(HEXA,HEXB)
    GM2AB       GM2A*
N02038    CTSA→(GLB1,NEU1)
    GSL/BSVD6 CTSA*
 
N01711    GA1−GLB1→GA2−(HEXA,HEXB)→LacCer
N01709        Gb4Cer−(HEXA,HEXB)→Gb3Cer−GLA→LacCer
    Fabry disease           GLA*
 
N02036    PSAP−Cathepsins→SAPA,SAPB,SAPC,SAP..
    PARK24/PSAPD PSAP*
    CLN10   CTSD*
    Pycnodysostosis   CTSK*
N02037    PGRN→CTSD
    FTD2/CLN11 GRN*
 
N00640    LacCer−GLB1→GlcCer−GBA→Ceramide
    GD       GBA*
N02039        SAPC→GBA
    GDSAPC     SAPC*
 
N00644    Ga2Cer−GLA→GalCer−GALC→Ceramide
N01712        Psychosine−GALC→Sphingosine
    KRB       GALC*
N02040        SAPA→GALC
    KRBSAPA     SAPA*
 
N00647    Sulfatide−ARSA→GalCer
    MLD   ARSA*
N02041    SAPB→ARSA
    MLDSAPB SAPB*
 
N00649    Sphingomyelin−SMPD→Ceramide−ASAH→Sphingosine
    NPD   SMPD1*
    FRBRL       ASAH1*
 
N02042    Glycogen−GAA→Glc
    GSDII   GAA*
 
N00610    DS−IDS⇉IDUA⇉ARSB⇉HYAL→G00872
    MPS2   IDS*
    MPS1     IDUA*
    MPS6       ARSB*
    MPS9         HYAL1*
 
N00615    HS−IDS⇉IDUA⇉SGSH⇉HGSNAT⇉NAGLU⇉GUSB⇉GNS→G02632
    MPS3A       SGSH*
    MPS3C/RP73         HGSNAT*
    MPS3B/CMT2V           NAGLU*
    MPS7             GUSB*
    MPS3D               GNS*
 
N00623    KS−GALNS⇉GLB1⇉GNS⇉HEXA/B→G01391
    MPS4A   GALNS*
    MPS4B     GLB1*
 
N02043    SUMF1→IDS,ARS,SGSH,GNS,G..
    MSD SUMF1*
 
N02044    G00018−FUCA1⇉AGA⇉ENGASE,CTBS⇉NEU1⇉GLB1⇉HEXA/B⇉MAN2B1⇉MANBA→GlcNAc+Mannose
    Fucosidosis   FUCA1*
    AGU     AGA*
    MANSA               MAN2B1*
    MANSB                 MANBA*
 
N02024    (DMXL+ROGDI+WDR7)=V1ATPase=VoATPase
    DFNA71/DEE81 DMXL2*
    KTZS ROGDI*
    ARCL2D/DEE93   ATP6V1A*
    DRTA2   ATP6V1B1*
    ZLS2/DOOD   ATP6V1B2*
    ARCL2C   ATP6V1E1*
    DEE104/NEDEBA     ATP6V0A1*
    ARCL2A/WSS     ATP6V0A2*
    OPTB1     TCIRG1*
    DRTA3     ATP6V0A4*
    EPEO3     ATP6V0C*
    IMD47/CDG2S     ATP6AP1*
N02045      PPT1→ATP6V0A1
    CLN1   PPT1*
N02025      H+(cyto),ATP−V-ATPase→H+(lyso),ADP
 
N01780    CE−LIPA→Cholesterol+FA
    CESD/WOLD   LIPA*
N02026        Cholesterol=NPC2=NPC1
    NPC2       NPC2*
N02027          Cholesterol(lyso)−NPC1+OSBPL5→Cholesterol(cyto)
    NPC1         NPC1*
N02028    Cholesterol(cyto)−ABCA2,ABCA5→Cholesterol(lyso)
    IDPOGSA   ABCA2*
    HTC3   ABCA5*
 
N02035    PI(3)P−(PIKFYVE+FIG4+VAC1..→PI(3,5)P2→mTORC1
    CFD   PIKFYVE*
    ALS11/CMT4J/BTOP/YVS   FIG4*
    SNDC   VAC14*
 
N02029    Ca2+(lyso)−MCOLN,TPC→Ca2+(cyto)
    MLIV/LECD   MCOLN1*
 
N02030    (Cl-)(lyso),(H+)(c..−CLC7,CLN7→(Cl-)(cyto),(H+)(l..
    OPTA2/B4   CLCN7*
    HOD   CLCN7*↗
    OPTB5   OSTM1*
    CLN7/CCMD   MFSD8*
 
N02031    Fe2+(lyso),Mn2+(ly..−NRAMP→Fe2+(cyto),Mn2+(cy..
      SLC11A1*
    AHMIO1   SLC11A2*
 
N02032    nucleoside(lyso)−SLC29A3→nucleoside(cyto)
    H syndrome   SLC29A3*
 
N02033    SA(lyso)−SLC17A5→SA(cyto)
    SD/ISSD   SLC17A5*
 
N02034    cystine(lyso)−CTNS→cystine(cyto)
    Cystinosis   CTNS*

Disease nameDisease category
GM1GH00281GM1 gangliosidosisInherited metabolic disorder, Lysosomal disease
TSDH02016Tay-Sachs diseaseInherited metabolic disorder, Lysosomal disease
SDH02017Sandhoff diseaseInherited metabolic disorder, Lysosomal disease
SialidosisH00142SialidosisInherited metabolic disorder, Lysosomal disease
GM2ABH00124GM2 gangliosidosesInherited metabolic disorder, Lysosomal disease
GSL/BSVD6H00276GalactosialidosisInherited metabolic disorder, Lysosomal disease
H00877Brain small vessel diseaseCardiovascular disease
Fabry diseaseH00125Fabry diseaseInherited metabolic disorder, Lysosomal disease
PARK24/PSAPDH00057Parkinson diseaseNeurodegenerative disease
H01239Combined SAP deficiencyInherited metabolic disorder, Lysosomal disease
CLN10H02279Neuronal ceroid lipofuscinosis due to Cathepsin D deficiencyInherited metabolic disorder, Lysosomal disease
PycnodysostosisH00273PycnodysostosisInherited metabolic disorder, Lysosomal disease
FTD2/CLN11H00078Frontotemporal lobar degenerationNeurodegenerative disease
H00149Neuronal ceroid lipofuscinosisInherited metabolic disorder, Lysosomal disease
GDH00126Gaucher diseaseInherited metabolic disorder, Lysosomal disease
GDSAPCH00126Gaucher diseaseInherited metabolic disorder, Lysosomal disease
KRBH00135Krabbe diseaseInherited metabolic disorder, Lysosomal disease
KRBSAPAH00135Krabbe diseaseInherited metabolic disorder, Lysosomal disease
MLDH00127Metachromatic leukodystrophyInherited metabolic disorder, Lysosomal disease
MLDSAPBH00127Metachromatic leukodystrophyInherited metabolic disorder, Lysosomal disease
NPDH00137Niemann-Pick disease type A/BInherited metabolic disorder, Lysosomal disease
FRBRLH00138Farber lipogranulomatosisInherited metabolic disorder, Lysosomal disease
GSDIIH01940Glycogen storage disease type IIInherited metabolic disorder, Lysosomal disease
MPS2H00129Mucopolysaccharidosis type IIInherited metabolic disorder, Lysosomal disease
MPS1H00128Mucopolysaccharidosis type IInherited metabolic disorder, Lysosomal disease
MPS6H00131Mucopolysaccharidosis type VIInherited metabolic disorder, Lysosomal disease
MPS9H00133Mucopolysaccharidosis type IXInherited metabolic disorder, Lysosomal disease
MPS3AH00130Mucopolysaccharidosis type IIIInherited metabolic disorder, Lysosomal disease
MPS3C/RP73H00130Mucopolysaccharidosis type IIIInherited metabolic disorder, Lysosomal disease
H00527Retinitis pigmentosaNervous system disease
MPS3B/CMT2VH00130Mucopolysaccharidosis type IIIInherited metabolic disorder, Lysosomal disease
H00264Charcot-Marie-Tooth diseaseNeurodegenerative disease
MPS7H00132Mucopolysaccharidosis type VIIInherited metabolic disorder, Lysosomal disease
MPS3DH00130Mucopolysaccharidosis type IIIInherited metabolic disorder, Lysosomal disease
MPS4AH00123Mucopolysaccharidosis type IVInherited metabolic disorder, Lysosomal disease
MPS4BH00123Mucopolysaccharidosis type IVInherited metabolic disorder, Lysosomal disease
MSDH00272Multiple sulfatase deficiencyInherited metabolic disorder, Lysosomal disease
FucosidosisH00141FucosidosisInherited metabolic disorder, Lysosomal disease
AGUH00145AspartylglucosaminuriaInherited metabolic disorder, Lysosomal disease
MANSAH00139alpha-MannosidosisInherited metabolic disorder, Lysosomal disease
MANSBH00140beta-MannosidosisInherited metabolic disorder, Lysosomal disease
DFNA71/DEE81H00604Deafness, autosomal dominantNervous system disease
H00606Early infantile epileptic encephalopathyNervous system disease
KTZSH02058Kohlschutter-Tonz syndromeNervous system disease
ARCL2D/DEE93H00557Cutis laxaCongenital malformation
H00606Early infantile epileptic encephalopathyNervous system disease
DRTA2H00428Distal renal tubular acidosisUrinary system disease
ZLS2/DOODH01573Zimmermann-Laband syndromeCongenital malformation
H02219DDOD syndromeCongenital malformation
ARCL2CH00557Cutis laxaCongenital malformation
DEE104/NEDEBAH00606Early infantile epileptic encephalopathyNervous system disease
H03040Neurodevelopmental disorder with epilepsy and brain atrophyCongenital malformation
ARCL2A/WSSH00557Cutis laxaCongenital malformation
OPTB1H00436OsteopetrosisCongenital malformation
DRTA3H00428Distal renal tubular acidosisUrinary system disease
EPEO3H02696Early-onset epilepsyNervous system disease
IMD47/CDG2SH00119Congenital disorders of glycosylation type IIInherited metabolic disorder
CLN1H02277Santavuori-Haltia diseaseInherited metabolic disorder, Lysosomal disease
CESD/WOLDH00148Lysosomal acid lipase deficiencyInherited metabolic disorder, Lysosomal disease
NPC2H00136Niemann-Pick disease type CInherited metabolic disorder, Lysosomal disease
NPC1H00136Niemann-Pick disease type CInherited metabolic disorder, Lysosomal disease
IDPOGSAH03046Intellectual developmental disorder with poor growth and with or without seizures or ataxiaCongenital malformation
HTC3H02417Gingival fibromatosis with hypertrichosisCongenital malformation
CFDH00957Corneal fleck dystrophyNervous system disease
ALS11/CMT4J/BTOP/YVSH00058Amyotrophic lateral sclerosis (ALS)Neurodegenerative disease
H00264Charcot-Marie-Tooth diseaseNeurodegenerative disease
H00271PolymicrogyriaCongenital malformation
H02127Yunis-Varon syndromeCongenital malformation
SNDCH02802Childhood-onset striatonigral degenerationNervous system disease
MLIV/LECDH00144Mucolipidosis IVInherited metabolic disorder, Lysosomal disease
H02822Lisch epithelial corneal dystrophyNervous system disease
OPTA2/B4H00436OsteopetrosisCongenital malformation
HODH03034Hypopigmentation, organomegaly, and delayed myelination and developmentCongenital malformation
OPTB5H00436OsteopetrosisCongenital malformation
CLN7/CCMDH00149Neuronal ceroid lipofuscinosisInherited metabolic disorder, Lysosomal disease
H01770Macular dystrophyNervous system disease
TBH00342TuberculosisBacterial infectious disease
AHMIO1H01196Hypochromic microcytic anemiaHematologic disease
H syndromeH00815H syndromeCongenital malformation
SD/ISSDH00147SialuriaInherited metabolic disorder, Lysosomal disease
CystinosisH00275CystinosisInherited metabolic disorder, Lysosomal disease