KEGG   PATHWAY: hsa00310Help
Entry
hsa00310                    Pathway                                

Name
Lysine degradation - Homo sapiens (human)
Class
Metabolism; Amino acid metabolism
BRITE hierarchy
Pathway map
Lysine degradation
hsa00310

All organismsOrtholog table
Module
Lysine degradation, lysine => saccharopine => acetoacetyl-CoA [PATH:hsa00310]
Disease
H00178  
Glutaric acidemia
H00188  
Hyperlysinemia
H00570  
Kabuki syndrome
H00718  
Sotos syndrome and Weaver syndrome
H00802  
Ehlers-Danlos syndrome (EDS)
H00907  
Kleefstra syndrome
H00980  
Nevo syndrome
H01192  
Lysyl hydroxylase 3 (LH3) deficiency
H01242  
Saccharopinuria
H01247  
Pyridoxine-dependent epilepsy (PDE)
H01364  
3-Hydroxyacyl-CoA dehydrogenase deficiency
Drug
D00123  
Cyanamide (JP17)
D00131  
Disulfiram (JP17/USP/INN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
10157  
AASS; aminoadipate-semialdehyde synthase [KO:K14157] [EC:1.5.1.9 1.5.1.8]
501  
ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.3 1.2.1.8 1.2.1.31]
51166  
AADAT; aminoadipate aminotransferase [KO:K00825] [EC:2.6.1.39 2.6.1.7]
55753  
OGDHL; oxoglutarate dehydrogenase-like [KO:K00164] [EC:1.2.4.2]
4967  
OGDH; oxoglutarate dehydrogenase [KO:K00164] [EC:1.2.4.2]
1743  
DLST; dihydrolipoamide S-succinyltransferase [KO:K00658] [EC:2.3.1.61]
2639  
GCDH; glutaryl-CoA dehydrogenase [KO:K00252] [EC:1.3.8.6]
3030  
HADHA; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1962  
EHHADH; enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:5.3.3.8 1.1.1.35 4.2.1.17]
1892  
ECHS1; enoyl-CoA hydratase, short chain, 1, mitochondrial [KO:K07511] [EC:4.2.1.17]
3033  
HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
39  
ACAT2; acetyl-CoA acetyltransferase 2 [KO:K00626] [EC:2.3.1.9]
38  
ACAT1; acetyl-CoA acetyltransferase 1 [KO:K00626] [EC:2.3.1.9]
123688  
HYKK; hydroxylysine kinase [KO:K18201] [EC:2.7.1.81]
85007  
PHYKPL; 5-phosphohydroxy-L-lysine phospho-lyase [KO:K18202] [EC:4.2.3.134]
51268  
PIPOX; pipecolic acid and sarcosine oxidase [KO:K00306] [EC:1.5.3.7 1.5.3.1]
55870  
ASH1L; ASH1 like histone lysine methyltransferase [KO:K06101] [EC:2.1.1.43]
84444  
DOT1L; DOT1 like histone lysine methyltransferase [KO:K11427] [EC:2.1.1.43]
10919  
EHMT2; euchromatic histone lysine methyltransferase 2 [KO:K11420] [EC:2.1.1.43]
79813  
EHMT1; euchromatic histone lysine methyltransferase 1 [KO:K11420] [EC:2.1.1.43]
64324  
NSD1; nuclear receptor binding SET domain protein 1 [KO:K15588] [EC:2.1.1.43]
7468  
WHSC1; Wolf-Hirschhorn syndrome candidate 1 [KO:K11424] [EC:2.1.1.43]
54904  
WHSC1L1; Wolf-Hirschhorn syndrome candidate 1-like 1 [KO:K11425] [EC:2.1.1.43]
23067  
SETD1B; SET domain containing 1B [KO:K11422] [EC:2.1.1.43]
9739  
SETD1A; SET domain containing 1A [KO:K11422] [EC:2.1.1.43]
29072  
SETD2; SET domain containing 2 [KO:K11423] [EC:2.1.1.43]
80854  
SETD7; SET domain containing lysine methyltransferase 7 [KO:K11431] [EC:2.1.1.43]
387893  
KMT5A; lysine methyltransferase 5A [KO:K11428] [EC:2.1.1.43]
9869  
SETDB1; SET domain bifurcated 1 [KO:K11421] [EC:2.1.1.43]
83852  
SETDB2; SET domain bifurcated 2 [KO:K18494] [EC:2.1.1.43]
6419  
SETMAR; SET domain and mariner transposase fusion gene [KO:K11433] [EC:2.1.1.43]
6839  
SUV39H1; suppressor of variegation 3-9 homolog 1 [KO:K11419] [EC:2.1.1.43]
79723  
SUV39H2; suppressor of variegation 3-9 homolog 2 [KO:K11419] [EC:2.1.1.43]
84787  
KMT5C; lysine methyltransferase 5C [KO:K11429] [EC:2.1.1.43]
51111  
KMT5B; lysine methyltransferase 5B [KO:K11429] [EC:2.1.1.43]
4297  
KMT2A; lysine methyltransferase 2A [KO:K09186] [EC:2.1.1.43]
8085  
KMT2D; lysine methyltransferase 2D [KO:K09187] [EC:2.1.1.43]
58508  
KMT2C; lysine methyltransferase 2C [KO:K09188] [EC:2.1.1.43]
9757  
KMT2B; lysine methyltransferase 2B [KO:K14959] [EC:2.1.1.43]
55904  
KMT2E; lysine methyltransferase 2E [KO:K09189] [EC:2.1.1.43]
79823  
CAMKMT; calmodulin-lysine N-methyltransferase [KO:K18826] [EC:2.1.1.60]
55217  
TMLHE; trimethyllysine hydroxylase, epsilon [KO:K00474] [EC:1.14.11.8]
217  
ALDH2; aldehyde dehydrogenase 2 family (mitochondrial) [KO:K00128] [EC:1.2.1.3]
224  
ALDH3A2; aldehyde dehydrogenase 3 family member A2 [KO:K00128] [EC:1.2.1.3]
219  
ALDH1B1; aldehyde dehydrogenase 1 family member B1 [KO:K00128] [EC:1.2.1.3]
223  
ALDH9A1; aldehyde dehydrogenase 9 family member A1 [KO:K00149] [EC:1.2.1.3 1.2.1.47]
8424  
BBOX1; gamma-butyrobetaine hydroxylase 1 [KO:K00471] [EC:1.14.11.1]
5351  
PLOD1; procollagen-lysine,2-oxoglutarate 5-dioxygenase 1 [KO:K00473] [EC:1.14.11.4]
5352  
PLOD2; procollagen-lysine,2-oxoglutarate 5-dioxygenase 2 [KO:K13645] [EC:1.14.11.4]
8985  
PLOD3; procollagen-lysine,2-oxoglutarate 5-dioxygenase 3 [KO:K13646] [EC:2.4.1.66 2.4.1.50 1.14.11.4]
79709  
COLGALT1; collagen beta(1-O)galactosyltransferase 1 [KO:K11703] [EC:2.4.1.50]
23127  
COLGALT2; collagen beta(1-O)galactosyltransferase 2 [KO:K11703] [EC:2.4.1.50]
Compound
C00024  
Acetyl-CoA
C00037  
Glycine
C00047  
L-Lysine
C00136  
Butanoyl-CoA
C00164  
Acetoacetate
C00322  
2-Oxoadipate
C00332  
Acetoacetyl-CoA
C00408  
L-Pipecolate
C00431  
5-Aminopentanoate
C00449  
N6-(L-1,3-Dicarboxypropyl)-L-lysine
C00450  
(S)-2,3,4,5-Tetrahydropyridine-2-carboxylate
C00487  
Carnitine
C00489  
Glutarate
C00527  
Glutaryl-CoA
C00739  
D-Lysine
C00877  
Crotonoyl-CoA
C00956  
L-2-Aminoadipate
C00990  
5-Aminopentanamide
C01028  
N6-Hydroxy-L-lysine
C01142  
(3S)-3,6-Diaminohexanoate
C01144  
(S)-3-Hydroxybutanoyl-CoA
C01149  
4-Trimethylammoniobutanal
C01181  
4-Trimethylammoniobutanoate
C01186  
(3S,5S)-3,5-Diaminohexanoate
C01211  
Procollagen 5-hydroxy-L-lysine
C01259  
3-Hydroxy-N6,N6,N6-trimethyl-L-lysine
C01672  
Cadaverine
C02188  
Protein lysine
C02727  
N6-Acetyl-L-lysine
C03087  
5-Acetamidopentanoate
C03239  
6-Amino-2-oxohexanoate
C03273  
5-Oxopentanoate
C03366  
5-Phosphonooxy-L-lysine
C03656  
(S)-5-Amino-3-oxohexanoic acid
C03793  
N6,N6,N6-Trimethyl-L-lysine
C03955  
N6-Acetyl-N6-hydroxy-L-lysine
C04020  
D-Lysopine
C04076  
L-2-Aminoadipate 6-semialdehyde
C04092  
delta1-Piperideine-2-carboxylate
C04487  
5-(D-Galactosyloxy)-L-lysine-procollagen
C05161  
2,5-Diaminohexanoate
C05231  
L-3-Aminobutyryl-CoA
C05544  
Protein N6-methyl-L-lysine
C05545  
Protein N6,N6-dimethyl-L-lysine
C05546  
Protein N6,N6,N6-trimethyl-L-lysine
C05548  
6-Acetamido-2-oxohexanoate
C05554  
Aerobactin
C05825  
2-Amino-5-oxohexanoate
C06157  
[Dihydrolipoyllysine-residue succinyltransferase] S-glutaryldihydrolipoyllysine
C06181  
Piperideine
C16741  
L-Hydroxylysine
C20333  
N2-Citryl-N6-acetyl-N6-hydroxy-L-lysine
Reference
  Authors
Vaz FM, Wanders RJ.
  Title
Carnitine biosynthesis in mammals.
  Journal
Biochem J 361:417-29 (2002)
Reference
PMID:1682209
  Authors
Large PJ, Robertson A.
  Title
The route of lysine breakdown in Candida tropicalis.
  Journal
FEMS Microbiol Lett 66:209-13 (1991)
KO pathway
 

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