KEGG   ORTHOLOGY: K12348
Entry
K12348                      KO                                     
Symbol
ASAH1
Name
acid ceramidase [EC:3.5.1.23]
Pathway
map00600  Sphingolipid metabolism
map01100  Metabolic pathways
map04071  Sphingolipid signaling pathway
map04142  Lysosome
Module
M00099  Sphingosine biosynthesis
Reaction
R01494  N-acylsphingosine amidohydrolase
R06518  N-acylsphingosine amidohydrolase
R06528  phytoceramide amidohydrolase
Disease
H00138  Farber lipogranulomatosis
H00423  Sphingolipidosis
H00424  Defects in the degradation of sphingomyelin
Brite
KEGG Orthology (KO) [BR:ko00001]
 09100 Metabolism
  09103 Lipid metabolism
   00600 Sphingolipid metabolism
    K12348  ASAH1; acid ceramidase
 09130 Environmental Information Processing
  09132 Signal transduction
   04071 Sphingolipid signaling pathway
    K12348  ASAH1; acid ceramidase
 09140 Cellular Processes
  09141 Transport and catabolism
   04142 Lysosome
    K12348  ASAH1; acid ceramidase
 09180 Brite Hierarchies
  09181 Protein families: metabolism
   01002 Peptidases and inhibitors
    K12348  ASAH1; acid ceramidase
  09183 Protein families: signaling and cellular processes
   04147 Exosome
    K12348  ASAH1; acid ceramidase
Enzymes [BR:ko01000]
 3. Hydrolases
  3.5  Acting on carbon-nitrogen bonds, other than peptide bonds
   3.5.1  In linear amides
    3.5.1.23  ceramidase
     K12348  ASAH1; acid ceramidase
Peptidases and inhibitors [BR:ko01002]
 Cysteine peptidases
  Family C89
   K12348  ASAH1; acid ceramidase
Exosome [BR:ko04147]
 Exosomal proteins
  Exosomal proteins of other body fluids (saliva and urine)
   K12348  ASAH1; acid ceramidase
Other DBs
GO: 0017040
Genes
HSA: 427(ASAH1)
PTR: 464022(ASAH1)
PPS: 100991999(ASAH1)
GGO: 101149522
PON: 100171626(ASAH1)
NLE: 100579536(ASAH1)
HMH: 116473765(ASAH1)
MCC: 703699(ASAH1)
MCF: 102118393(ASAH1)
MTHB: 126961451
MNI: 105463728(ASAH1)
CSAB: 103215400(ASAH1)
CATY: 105601404(ASAH1)
PANU: 101008527(ASAH1)
TGE: 112630412(ASAH1)
MLEU: 105539387(ASAH1)
RRO: 104655223(ASAH1)
RBB: 108526474(ASAH1)
TFN: 117092723(ASAH1)
PTEH: 111545656(ASAH1)
CANG: 105506653(ASAH1)
CJC: 100409902(ASAH1)
SBQ: 101044863(ASAH1)
CIMI: 108311996(ASAH1)
CSYR: 103263195(ASAH1)
MMUR: 105882521(ASAH1)
LCAT: 123626488(ASAH1)
PCOQ: 105825204(ASAH1)
OGA: 100944759(ASAH1)
MMU: 11886(Asah1)
MCAL: 110299751(Asah1)
MPAH: 110336346(Asah1)
RNO: 84431(Asah1)
MCOC: 116069197(Asah1)
ANU: 117702895 117721530(Asah1)
MUN: 110558780(Asah1)
CGE: 100771068(Asah1)
MAUA: 101840880(Asah1)
PROB: 127220449(Asah1)
PLEU: 114695135(Asah1)
MORG: 121442819(Asah1)
MFOT: 126504764
AAMP: 119811309(Asah1)
NGI: 103748255(Asah1)
HGL: 101724913(Asah1)
CPOC: 100731092 100735549(Asah1)
CCAN: 109692105(Asah1)
DORD: 105993070(Asah1)
DSP: 122125834(Asah1)
PLOP: 125339342(Asah1)
NCAR: 124983780
OCU: 100338209
OPI: 101531935(ASAH1)
TUP: 102490603(ASAH1)
GVR: 103590191(ASAH1)
CFA: 482897(ASAH1)
CLUD: 112672246(ASAH1)
VVP: 112908911(ASAH1)
VLG: 121489218(ASAH1)
NPO: 129515863(ASAH1)
AML: 100471625(ASAH1)
UMR: 103681376(ASAH1)
UAH: 113262148(ASAH1)
UAR: 123780430(ASAH1)
ELK: 111144609
LLV: 125092241
MPUF: 101689409(ASAH1)
MNP: 132006334(ASAH1)
NVS: 122889922(ASAH1)
ORO: 101384361(ASAH1)
EJU: 114206151(ASAH1)
ZCA: 113925026(ASAH1)
MLX: 118010040(ASAH1)
NSU: 110584178(ASAH1)
LWW: 102745505(ASAH1)
FCA: 101082231(ASAH1)
PYU: 121039724(ASAH1)
PCOO: 112869995(ASAH1)
PBG: 122471391(ASAH1)
LRUF: 124520027
PTG: 102969154(ASAH1)
PPAD: 109277196(ASAH1)
PUC: 125923438
AJU: 106988336
HHV: 120248089(ASAH1)
BTA: 510620(ASAH1)
BOM: 102285015(ASAH1)
BIU: 109553501(ASAH1)
BBUB: 102414906(ASAH1)
BBIS: 105001915(ASAH1)
CHX: 102169733(ASAH1)
OAS: 101117904(ASAH1)
BTAX: 128068307(ASAH1)
ODA: 120865766(ASAH1)
CCAD: 122432358(ASAH1)
MBEZ: 129549335(ASAH1)
SSC: 100512932(ASAH1)
CFR: 102503963(ASAH1)
CBAI: 105067660(ASAH1)
CDK: 105102375(ASAH1)
VPC: 102527346(ASAH1)
BACU: 103004383(ASAH1)
LVE: 103084758(ASAH1)
OOR: 101276356(ASAH1)
DLE: 111173434(ASAH1)
PCAD: 102977037(ASAH1)
PSIU: 116746719(ASAH1)
NASI: 112409655(ASAH1)
EPZ: 103556309(ASAH1)
EAI: 106828200(ASAH1)
MYB: 102261355(ASAH1)
MYD: 102772524(ASAH1)
MMYO: 118650186(ASAH1)
MLF: 102425151(ASAH1)
MNA: 107534040(ASAH1)
PKL: 118730877(ASAH1)
EFUS: 103290363(ASAH1)
HAI: 109394586(ASAH1)
DRO: 112306675(ASAH1)
SHON: 118993101(ASAH1)
AJM: 119036836(ASAH1)
PDIC: 114508612(ASAH1)
PHAS: 123807488(ASAH1)
MMF: 118622677(ASAH1)
RFQ: 117021668(ASAH1)
PPAM: 129064868(ASAH1)
PALE: 102884758(ASAH1)
PGIG: 120583819(ASAH1)
PVP: 105295539(ASAH1)
RAY: 107502652(ASAH1)
MJV: 108396511(ASAH1)
TOD: 119257925(ASAH1)
SARA: 101555645(ASAH1)
LAV: 100658494(ASAH1)
TMU: 101359387
ETF: 101659974(ASAH1)
DNM: 101437929(ASAH1)
MDO: 100021204(ASAH1)
GAS: 123251812(ASAH1)
SHR: 100927628(ASAH1)
AFZ: 127540827
PCW: 110201225(ASAH1)
OAA: 100093499(ASAH1)
GGA: 422727(ASAH1)
PCOC: 116225949(ASAH1)
MGP: 100550236(ASAH1)
CJO: 107313735(ASAH1)
TPAI: 128080156(ASAH1)
LMUT: 125692259(ASAH1)
NMEL: 110397955(ASAH1)
APLA: 101801719(ASAH1)
ACYG: 106035211(ASAH1)
CATA: 118245477(ASAH1)
AFUL: 116488772(ASAH1)
TGU: 100223185(ASAH1)
LSR: 110474653(ASAH1)
SCAN: 103826204(ASAH1)
PMOA: 120497969(ASAH1)
OTC: 121335167(ASAH1)
PRUF: 121360282(ASAH1)
GFR: 102045042(ASAH1)
FAB: 101806040(ASAH1)
OMA: 130252161(ASAH1)
PHI: 102108790(ASAH1)
PMAJ: 107203488(ASAH1)
CCAE: 111928436(ASAH1)
CCW: 104690859(ASAH1)
CBRC: 103622566(ASAH1)
ETL: 114064485(ASAH1)
ZAB: 102065751(ASAH1)
ACHL: 103803226(ASAH1)
SVG: 106851496(ASAH1)
MMEA: 130583162(ASAH1)
HRT: 120753286(ASAH1)
FPG: 101917300(ASAH1)
FCH: 102045851(ASAH1)
CLV: 102098583(ASAH1)
EGZ: 104129441(ASAH1)
NNI: 104020838(ASAH1)
PCRI: 104029022(ASAH1)
PLET: 104621869(ASAH1)
EHS: 104514540(ASAH1)
PCAO: 104041030(ASAH1)
ACUN: 113479231(ASAH1)
TALA: 104360125(ASAH1)
PADL: 103926331(ASAH1)
AFOR: 103897420(ASAH1)
ACHC: 115340599(ASAH1)
HALD: 104324489(ASAH1)
HLE: 104833399(ASAH1)
AGEN: 126037467
GCL: 127015840
CCRI: 104167847(ASAH1)
CSTI: 104561835(ASAH1)
CMAC: 104473886(ASAH1)
MUI: 104540016(ASAH1)
BREG: 104643443(ASAH1)
FGA: 104070657(ASAH1)
GSTE: 104251160(ASAH1)
LDI: 104340545(ASAH1)
MNB: 103773396(ASAH1)
OHA: 104334940(ASAH1)
NNT: 104408606(ASAH1)
SHAB: 115610330(ASAH1)
DPUB: 104304171(ASAH1)
PGUU: 104467541
ACAR: 104528140(ASAH1)
CPEA: 104393050(ASAH1)
AVIT: 104277269(ASAH1)
CVF: 104285457(ASAH1)
RTD: 128910408(ASAH1)
CUCA: 104067121(ASAH1)
TEO: 104379550(ASAH1)
BRHI: 104501527(ASAH1)
AAM: 106490075(ASAH1)
AROW: 112969367(ASAH1)
NPD: 112942600(ASAH1)
TGT: 104576875(ASAH1)
DNE: 112985735(ASAH1)
SCAM: 104138342(ASAH1)
ASN: 102383990(ASAH1)
AMJ: 102566045(ASAH1)
CPOO: 109321837(ASAH1)
GGN: 109303899(ASAH1)
PSS: 102444687(ASAH1)
CMY: 102930137(ASAH1)
CCAY: 125635857(ASAH1)
DCC: 119854764(ASAH1)
CPIC: 101944232(ASAH1)
TST: 117877806(ASAH1)
CABI: 116835381(ASAH1)
MRV: 120405914(ASAH1)
ACS: 100565312(asah1)
PVT: 110077885(ASAH1)
SUND: 121931689(ASAH1)
PBI: 103052913(ASAH1)
PMUR: 107301677(ASAH1)
CTIG: 120310692(ASAH1)
TSR: 106554883(ASAH1)
PGUT: 117654607(ASAH1)
APRI: 131202925(ASAH1)
PTEX: 113434659(ASAH1)
NSS: 113411838(ASAH1)
VKO: 123036229(ASAH1)
PMUA: 114603838(ASAH1)
PRAF: 128420754(ASAH1)
ZVI: 118083625(ASAH1)
HCG: 128326835(ASAH1)
GJA: 107108734(ASAH1)
STOW: 125439974(ASAH1)
EMC: 129333092 129337019(ASAH1)
XLA: 447327(asah1.L)
XTR: 100485700(asah1)
NPR: 108785692(ASAH1)
RTEM: 120921971(ASAH1)
BBUF: 120990168(ASAH1)
BGAR: 122942236(ASAH1)
GSH: 117360289(ASAH1)
DRE: 393549(asah1b) 450068(asah1a)
PTET: 122349984(asah1b) 122357205(asah1a)
LROH: 127158047(asah1b) 127176682(asah1a)
OMC: 131553380(asah1a) 131553986(asah1b)
PPRM: 120474977(asah1a) 120481366(asah1b)
MAMB: 125259353(asah1a) 125272545(asah1b)
TROS: 130552693(asah1b) 130563704(asah1a)
MANU: 129422762(asah1a) 129444797(asah1b)
IPU: 108260777(asah1b) 108269357(asah1a)
IFU: 128604440(asah1b) 128611329(asah1a)
PHYP: 113528763 113547387(asah1)
SMEO: 124381592(asah1b) 124386636(asah1a)
TFD: 113635536(asah1b) 113635578(asah1a)
AMEX: 103026123(asah1) 103040700
CMAO: 118806945(asah1a) 118816948(asah1b)
CHAR: 105892590(asah1b) 105911479
TRU: 101065149(asah1)
TFS: 130527986(asah1b)
LCO: 104936951(asah1)
NCC: 104967119(asah1)
CGOB: 115008953(asah1)
ELY: 117256061(asah1b)
EFO: 125885453(asah1b)
PLEP: 121942595(asah1b)
SLUC: 116034896(asah1b)
ECRA: 117959847(asah1b)
ESP: 116670217(asah1)
PFLV: 114546501(asah1)
GAT: 120824959(asah1b)
PPUG: 119218605(asah1b)
AFB: 129095951(asah1b)
CLUM: 117737830(asah1b)
MSAM: 119898805(asah1b)
SCHU: 122873699(asah1b)
CUD: 121509127(asah1b)
ALAT: 119010594(asah1b)
MZE: 101476420(asah1)
ONL: 100703085(asah1)
OAU: 116334010(asah1b)
OLA: 101161703(asah1)
OML: 112137240(asah1b)
XMA: 102231620(asah1)
XCO: 114144122(asah1)
XHE: 116719553(asah1)
PRET: 103461639(asah1)
PFOR: 103136381(asah1)
PLAI: 106938286(asah1)
PMEI: 106933338(asah1)
GAF: 122830172(asah1b)
CVG: 107090456(asah1)
CTUL: 119774384(asah1b)
GMU: 124868755(asah1b)
NFU: 107373323(asah1)
KMR: 108233727(asah1b)
ALIM: 106525838(asah1)
NWH: 119423059(asah1b)
AOCE: 111580238(asah1)
MCEP: 125001284(asah1b)
CSEM: 103384334(asah1)
POV: 109639561(asah1)
SSEN: 122771660(asah1b)
HHIP: 117770435(asah1b)
HSP: 118116651(asah1b)
SDU: 111216721(asah1)
SLAL: 111663285(asah1)
XGL: 120800266(asah1b)
HCQ: 109511169(asah1)
SSCV: 125969327
BPEC: 110167588(asah1)
MALB: 109972118(asah1)
BSPL: 114858833(asah1b)
CCLU: 123487891
ELS: 105022854(asah1)
SFM: 108924408(asah1)
PKI: 111853589(asah1)
AANG: 118228465(asah1b)
LOC: 102693609(asah1)
ARUT: 117408590(asah1b)
PSEX: 120527489(asah1b)
LCM: 102355600(ASAH1)
CMK: 103178195(asah1b)
RTP: 109929941(asah1)
CPLA: 122549810(asah1b)
LERI: 129697062(asah1b)
BFO: 118420272
BBEL: 109487765
CIN: 100179893
AJC: 117100869
SKO: 102808956
BTAB: 109038589
DCI: 103518434
HVI: 124357507
FOC: 113203190
TPAL: 117651021
IEL: 124163084
FCD: 110859289
DMK: 116917926
DPZ: 124344579
HAZT: 108664565
DSV: 119449998
RSAN: 119383007
RMP: 119170212
VDE: 111244233
VJA: 111258634
CSCU: 111623012
LPOL: 106478798
PMEO: 129584765
CEL: CELE_F27E5.1(asah-2) CELE_K11D2.2(asah-1)
CBR: CBG_00863 CBG_15381(Cbr-asah-1) CBG_15382
TSP: Tsp_09460
GAE: 121374062
CRG: 105334663
MYI: 110457570
PMAX: 117328790
MCAF: 127714911
OBI: 106876330
OSN: 115209837
NVE: 5506073
EPA: 110240322
ATEN: 116286612
ADF: 107356522
AMIL: 114955375
DFA: DFA_06303
 » show all
Reference
PMID:8955159
  Authors
Koch J, Gartner S, Li CM, Quintern LE, Bernardo K, Levran O, Schnabel D, Desnick RJ, Schuchman EH, Sandhoff K
  Title
Molecular cloning and characterization of a full-length complementary DNA encoding human acid ceramidase. Identification Of the first molecular lesion causing Farber disease.
  Journal
J Biol Chem 271:33110-5 (1996)
DOI:10.1074/jbc.271.51.33110
  Sequence
[hsa:427]

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