KEGG   ORTHOLOGY: K12657Help
Entry
K12657                      KO                                     

Name
ALDH18A1, P5CS
Definition
delta-1-pyrroline-5-carboxylate synthetase [EC:2.7.2.11 1.2.1.41]
Pathway
Arginine and proline metabolism
Biosynthesis of amino acids
Module
M00015  
Proline biosynthesis, glutamate => proline
Disease
H00557  
Cutis laxa
H01400  
Secondary hyperammonemia
Brite
KEGG Orthology (KO) [BR:ko00001]
 Metabolism
  Overview
   01230 Biosynthesis of amino acids
    K12657  ALDH18A1, P5CS; delta-1-pyrroline-5-carboxylate synthetase
  Amino acid metabolism
   00330 Arginine and proline metabolism
    K12657  ALDH18A1, P5CS; delta-1-pyrroline-5-carboxylate synthetase
KEGG modules [BR:ko00002]
 Pathway module
  Nucleotide and amino acid metabolism
   Arginine and proline metabolism
    M00015  Proline biosynthesis, glutamate => proline
     K12657  ALDH18A1, P5CS; delta-1-pyrroline-5-carboxylate synthetase
Enzymes [BR:ko01000]
 1. Oxidoreductases
  1.2  Acting on the aldehyde or oxo group of donors
   1.2.1  With NAD+ or NADP+ as acceptor
    1.2.1.41  glutamate-5-semialdehyde dehydrogenase
     K12657  ALDH18A1, P5CS; delta-1-pyrroline-5-carboxylate synthetase
 2. Transferases
  2.7  Transferring phosphorus-containing groups
   2.7.2  Phosphotransferases with a carboxy group as acceptor
    2.7.2.11  glutamate 5-kinase
     K12657  ALDH18A1, P5CS; delta-1-pyrroline-5-carboxylate synthetase
BRITE hierarchy
Other DBs
Genes
HSA: 
5832(ALDH18A1)
PTR: 
450629(ALDH18A1)
PPS: 
100969924(ALDH18A1)
GGO: 
101130918(ALDH18A1)
PON: 
100173488(ALDH18A1)
NLE: 
100592145(ALDH18A1)
MCC: 
703357(ALDH18A1)
MCF: 
102131075(ALDH18A1)
CJC: 
100394646(ALDH18A1)
MMU: 
56454(Aldh18a1)
RNO: 
361755(Aldh18a1)
CGE: 
100755485(Aldh18a1)
HGL: 
101713177(Aldh18a1)
TUP: 
102470903(ALDH18A1)
CFA: 
477781(ALDH18A1)
AML: 
UMR: 
103671341(ALDH18A1)
FCA: 
101090803(ALDH18A1)
PTG: 
102958449(ALDH18A1)
BTA: 
514759(ALDH18A1)
BOM: 
102269374(ALDH18A1)
PHD: 
102322285(ALDH18A1)
CHX: 
102188107(ALDH18A1)
OAS: 
101108974(ALDH18A1)
SSC: 
100151977(ALDH18A1)
CFR: 
102523778(ALDH18A1)
BACU: 
103016461(ALDH18A1)
LVE: 
103083754(ALDH18A1)
ECB: 
100061213(ALDH18A1)
MYB: 
102257395(ALDH18A1)
MYD: 
102753368(ALDH18A1)
PALE: 
102891029(ALDH18A1)
MDO: 
100023017(ALDH18A1)
SHR: 
100933253(ALDH18A1)
OAA: 
100077152(ALDH18A1)
GGA: 
423976(ALDH18A1)
MGP: 
APLA: 
101804818(ALDH18A1)
TGU: 
FAB: 
101810669(ALDH18A1)
PHI: 
102099899(ALDH18A1)
FPG: 
101922420(ALDH18A1)
FCH: 
102053636(ALDH18A1)
CLV: 
102091714(ALDH18A1)
ASN: 
102372617(ALDH18A1)
PSS: 
CMY: 
ACS: 
100563206(aldh18a1)
PBI: 
103052669(ALDH18A1)
XLA: 
444469(aldh18a1)
XTR: 
550060(aldh18a1)
DRE: 
100332705 557186(aldh18a1)
TRU: 
MZE: 
OLA: 
XMA: 
LCM: 
CMK: 
103189972(aldh18a1)
BFO: 
CIN: 
SPU: 
DME: 
DPO: 
DAN: 
DER: 
DPE: 
DSE: 
DSI: 
DWI: 
DYA: 
DGR: 
DMO: 
DVI: 
AGA: 
AAG: 
CQU: 
AME: 
412948(GB15049)
NVI: 
TCA: 
BMOR: 
API: 
PHU: 
ISC: 
CEL: 
CELE_T22H6.2(alh-13)
CBR: 
TSP: 
LGI: 
SMM: 
NVE: 
HMG: 
AQU: 
ATH: 
AT2G39800(P5CS1) AT3G55610(P5CS2)
ALY: 
CRB: 
EUS: 
CIT: 
CIC: 
TCC: 
GMX: 
PVU: 
MTR: 
CAM: 
FVE: 
PPER: 
PMUM: 
MDM: 
CSV: 
CMO: 
RCU: 
POP: 
VVI: 
SLY: 
SOT: 
OSA: 
DOSA: 
Os01t0848200-01(Os01g0848200) Os05t0455500-01(Os05g0455500)
OBR: 
BDI: 
SBI: 
SORBI_09g022310(SORBIDRAFT_09g022310)
ZMA: 
100280719(pco085448)
SITA: 
ATR: 
s00012p00263260(AMTR_s00012p00263260)
SMO: 
PPP: 
CRE: 
VCN: 
OTA: 
BPG: 
MIS: 
MPP: 
CSL: 
CVR: 
MBR: 
ACAN: 
PTI: 
TPS: 
PIF: 
GTT: 
 » show all
TaxonomyKoalaUniProt
Reference
  Authors
Baumgartner MR, Hu CA, Almashanu S, Steel G, Obie C, Aral B, Rabier D, Kamoun P, Saudubray JM, Valle D
  Title
Hyperammonemia with reduced ornithine, citrulline, arginine and proline: a new inborn error caused by a mutation in the gene encoding delta(1)-pyrroline-5-carboxylate synthase.
  Journal
Hum Mol Genet 9:2853-8 (2000)
  Sequence
[hsa:5832]
Reference
  Authors
Hu CA, Lin WW, Obie C, Valle D
  Title
Molecular enzymology of mammalian Delta1-pyrroline-5-carboxylate synthase. Alternative splice donor utilization generates isoforms with different sensitivity to ornithine inhibition.
  Journal
J Biol Chem 274:6754-62 (1999)
  Sequence
[hsa:5832]

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