KEGG   PATHWAY: hsa00310Help
Entry
hsa00310                    Pathway                                

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

Ortholog table
Module
hsa_M00032  Lysine degradation, lysine => saccharopine => acetoacetyl-CoA [PATH:hsa00310]
Disease
H00178  Glutaric acidemia
H00188  Hyperlysinemia
H00570  Kabuki syndrome
H00718  Sotos syndrome
H00907  Kleefstra syndrome
H00980  Nevo syndrome
H01192  Lysyl hydroxylase 3 deficiency
H01242  Saccharopinuria
H01247  Pyridoxine-dependent epilepsy
H01364  3-Hydroxyacyl-CoA dehydrogenase deficiency
H01751  Weaver syndrome
Drug
D11444  Tazemetostat (USAN/INN)
D11485  Tazemetostat hydrobromide (JAN/USAN)
D11551  Valemetostat
D11662  Valemetostat tosilate (JAN)
Organism
Homo sapiens (human) [GN:hsa]
Gene
10157  AASS; aminoadipate-semialdehyde synthase [KO:K14157] [EC:1.5.1.8 1.5.1.9]
501  ALDH7A1; aldehyde dehydrogenase 7 family member A1 [KO:K14085] [EC:1.2.1.31 1.2.1.8 1.2.1.3]
51166  AADAT; aminoadipate aminotransferase [KO:K00825] [EC:2.6.1.7 2.6.1.39]
55526  DHTKD1; dehydrogenase E1 and transketolase domain containing 1 [KO:K15791] [EC:1.2.4.2]
1743  DLST; dihydrolipoamide S-succinyltransferase [KO:K00658] [EC:2.3.1.61]
1738  DLD; dihydrolipoamide dehydrogenase [KO:K00382] [EC:1.8.1.4]
2639  GCDH; glutaryl-CoA dehydrogenase [KO:K00252] [EC:1.3.8.6]
3030  HADHA; hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha [KO:K07515] [EC:4.2.1.17 1.1.1.211]
1962  EHHADH; enoyl-CoA hydratase and 3-hydroxyacyl CoA dehydrogenase [KO:K07514] [EC:4.2.1.17 1.1.1.35 5.3.3.8]
1892  ECHS1; enoyl-CoA hydratase, short chain 1 [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:1.5.3.1 1.5.3.7]
79823  CAMKMT; calmodulin-lysine N-methyltransferase [KO:K18826] [EC:2.1.1.60]
4297  KMT2A; lysine methyltransferase 2A [KO:K09186] [EC:2.1.1.354]
8085  KMT2D; lysine methyltransferase 2D [KO:K09187] [EC:2.1.1.354]
58508  KMT2C; lysine methyltransferase 2C [KO:K09188] [EC:2.1.1.354]
9757  KMT2B; lysine methyltransferase 2B [KO:K14959] [EC:2.1.1.354]
55904  KMT2E; lysine methyltransferase 2E [KO:K09189] [EC:2.1.1.354]
23067  SETD1B; SET domain containing 1B, histone lysine methyltransferase [KO:K11422] [EC:2.1.1.354]
9739  SETD1A; SET domain containing 1A, histone lysine methyltransferase [KO:K11422] [EC:2.1.1.354]
80854  SETD7; SET domain containing 7, histone lysine methyltransferase [KO:K11431] [EC:2.1.1.354]
56979  PRDM9; PR/SET domain 9 [KO:K20796] [EC:2.1.1.354]
11105  PRDM7; PR/SET domain 7 [KO:K20796] [EC:2.1.1.354]
55870  ASH1L; ASH1 like histone lysine methyltransferase [KO:K06101] [EC:2.1.1.354]
150572  SMYD1; SET and MYND domain containing 1 [KO:K11426] [EC:2.1.1.354 2.1.1.357]
56950  SMYD2; SET and MYND domain containing 2 [KO:K11426] [EC:2.1.1.354 2.1.1.357]
64754  SMYD3; SET and MYND domain containing 3 [KO:K11426] [EC:2.1.1.354 2.1.1.357]
6839  SUV39H1; suppressor of variegation 3-9 homolog 1 [KO:K11419] [EC:2.1.1.355]
79723  SUV39H2; suppressor of variegation 3-9 homolog 2 [KO:K11419] [EC:2.1.1.355]
10919  EHMT2; euchromatic histone lysine methyltransferase 2 [KO:K11420] [EC:2.1.1.355]
79813  EHMT1; euchromatic histone lysine methyltransferase 1 [KO:K11420] [EC:2.1.1.355]
9869  SETDB1; SET domain bifurcated histone lysine methyltransferase 1 [KO:K11421] [EC:2.1.1.355]
83852  SETDB2; SET domain bifurcated histone lysine methyltransferase 2 [KO:K18494] [EC:2.1.1.355]
7799  PRDM2; PR/SET domain 2 [KO:K11432] [EC:2.1.1.355]
2145  EZH1; enhancer of zeste 1 polycomb repressive complex 2 subunit [KO:K17451] [EC:2.1.1.356]
2146  EZH2; enhancer of zeste 2 polycomb repressive complex 2 subunit [KO:K11430] [EC:2.1.1.356]
64324  NSD1; nuclear receptor binding SET domain protein 1 [KO:K15588] [EC:2.1.1.357]
7468  NSD2; nuclear receptor binding SET domain protein 2 [KO:K11424] [EC:2.1.1.357]
54904  NSD3; nuclear receptor binding SET domain protein 3 [KO:K11425] [EC:2.1.1.357]
6419  SETMAR; SET domain and mariner transposase fusion gene [KO:K11433] [EC:2.1.1.357]
29072  SETD2; SET domain containing 2, histone lysine methyltransferase [KO:K11423] [EC:2.1.1.358]
84444  DOT1L; DOT1 like histone lysine methyltransferase [KO:K11427] [EC:2.1.1.360]
387893  KMT5A; lysine methyltransferase 5A [KO:K11428] [EC:2.1.1.361]
93166  PRDM6; PR/SET domain 6 [KO:K20795] [EC:2.1.1.361]
84787  KMT5C; lysine methyltransferase 5C [KO:K11429] [EC:2.1.1.362]
51111  KMT5B; lysine methyltransferase 5B [KO:K11429] [EC:2.1.1.362]
55217  TMLHE; trimethyllysine hydroxylase, epsilon [KO:K00474] [EC:1.14.11.8]
217  ALDH2; aldehyde dehydrogenase 2 family member [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:1.2.1.47 1.2.1.3]
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:1.14.11.4 2.4.1.50 2.4.1.66]
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
C00026  2-Oxoglutarate
C00037  Glycine
C00042  Succinate
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  (3S)-3-Hydroxy-N6,N6,N6-trimethyl-L-lysine
C01672  Cadaverine
C02188  Protein lysine
C02727  N6-Acetyl-L-lysine
C03087  5-Acetamidopentanoate
C03196  (S)-2-Hydroxyglutarate
C03239  6-Amino-2-oxohexanoate
C03273  5-Oxopentanoate
C03366  5-Phosphooxy-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  (2R,5S)-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
C12455  5-Aminopentanal
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)
DOI:10.1042/bj3610417
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)
DOI:10.1111/j.1574-6968.1990.tb03998.x
KO pathway
ko00310   

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