Sequence of P5CS_HUMAN
EC Number:1.2.1.41
EC Number
Recommended Name
Accession Code
Organism
No of amino acids
Molecular Weight [Da]
Source
Reaction
L-glutamyl 5-phosphate + NADPH + H+ = L-glutamate 5-semialdehyde + phosphate + NADP+
Other sequences found for EC No. 1.2.1.41
General information:
Sequence
0 MLSQVYRCGF QPFNQHLLPW VKCTTVFRSH CIQPSVIRHV RSWSNIPFIT VPLSRTHGKS
60 FAHRSELKHA KRIVVKLGSA VVTRGDECGL ALGRLASIVE QVSVLQNQGR EMMLVTSGAV
120 AFGKQRLRHE ILLSQSVRQA LHSGQNQLKE MAIPVLEARA CAAAGQSGLM ALYEAMFTQY
180 SICAAQILVT NLDFHDEQKR RNLNGTLHEL LRMNIVPIVN TNDAVVPPAE PNSDLQGVNV
240 ISVKDNDSLA ARLAVEMKTD LLIVLSDVEG LFDSPPGSDD AKLIDIFYPG DQQSVTFGTK
300 SRVGMGGMEA KVKAALWALQ GGTSVVIANG THPKVSGHVI TDIVEGKKVG TFFSEVKPAG
360 PTVEQQGEMA RSGGRMLATL EPEQRAEIIH HLADLLTDQR DEILLANKKD LEEAEGRLAA
420 PLLKRLSLST SKLNSLAIGL RQIAASSQDS VGRVLRRTRI AKNLELEQVT VPIGVLLVIF
480 ESRPDCLPQV AALAIASGNG LLLKGGKEAA HSNRILHLLT QEALSIHGVK EAVQLVNTRE
540 EVEDLCRLDK MIDLIIPRGS SQLVRDIQKA AKGIPVMGHS EGICHMYVDS EASVDKVTRL
600 VRDSKCEYPA ACNALETLLI HRDLLRTPLF DQIIDMLRVE QVKIHAGPKF ASYLTFSPSE
660 VKSLRTEYGD LELCIEVVDN VQDAIDHIHK YGSSHTDVIV TEDENTAEFF LQHVDSACVF
720 WNASTRFSDG YRFGLGAEVG ISTSRIHARG PVGLEGLLTT KWLLRGKDHV VSDFSEHGSL
780 KYLHENLPIP QRNTN
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Sequence Reference
Authors
Title
Journal
Volume
Pages
Year
PubMed ID
71089
Aral B.,Schlenzig J.S.,Liu G.,Kamoun P.
Database cloning human delta 1-pyrroline-5-carboxylate synthetase (P5CS) cDNA: a bifunctional enzyme catalyzing the first 2 steps in proline biosynthesis.
C. R. Acad. Sci. III, Sci. Vie
319
171-178
1996
71090
Hu C.A.,Lin W.-W.,Obie C.,Valle D.
Molecular enzymology of mammalian delta1-pyrroline-5-carboxylate synthase. Alternative splice donor utilization generates isoforms with different sensitivity to ornithine inhibition.
J. Biol. Chem.
274
6754-6762
1999
71091
Ota T.,Suzuki Y.,Nishikawa T.,Otsuki T.,Sugiyama T.,Irie R.,Wakamatsu A.,Hayashi K.,Sato H.,Nagai K.,Kimura K.,Makita H.,Sekine M.,Obayashi M.,Nishi T.,Shibahara T.,Tanaka T.,Ishii S.,Yamamoto J.,Saito K.,Kawai Y.,Isono Y.,Nakamura Y.,Nagahari K.,Murakami K.,Yasuda T.,Iwayanagi T.,Wagatsuma M.,Shiratori A.,Sudo H.,Hosoiri T.,Kaku Y.,Kodaira H.,Kondo H.,Sugawara M.,Takahashi M.,Kanda K.,Yokoi T.,Furuya T.,Kikkawa E.,Omura Y.,Abe K.,Kamihara K.,Katsuta N.,Sato K.,Tanikawa M.,Yamazaki M.,Ninomiya K.,Ishibashi T.,Yamashita H.,Murakawa K.,Fujimori K.,Tanai H.,Kimata M.,Watanabe M.,Hiraoka S.,Chiba Y.,Ishida S.,Ono Y.,Takiguchi S.,Watanabe S.,Yosida M.,Hotuta T.,Kusano J.,Kanehori K.,Takahashi-Fujii A.,Hara H.,Tanase T.-O.,Nomura Y.,Togiya S.,Komai F.,Hara R.,Takeuchi K.,Arita M.,Imose N.,Musashino K.,Yuuki H.,Oshima A.,Sasaki N.,Aotsuka S.,Yoshikawa Y.,Matsunawa H.,Ichihara T.,Shiohata N.,Sano S.,Moriya S.,Momiyama H.,Satoh N.,Takami S.,Terashima Y.,Suzuki O.,Nakagawa S.,Senoh A.,Mizoguchi H.,Goto Y.,Shimizu F.,Wakebe H.,Hishigaki H.,Watanabe T.,Sugiyama A.,Takemoto M.,Kawakami B.,Yamazaki M.,Watanabe K.,Kumagai A.,Itakura S.,Fukuzumi Y.,Fujimori Y.,Komiyama M.,Tashiro H.,Tanigami A.,Fujiwara T.,Ono T.,Yamada K.,Fujii Y.,Ozaki K.,Hirao M.,Ohmori Y.,Kawabata A.,Hikiji T.,Kobatake N.,Inagaki H.,Ikema Y.,Okamoto S.,Okitani R.,Kawakami T.,Noguchi S.,Itoh T.,Shigeta K.,Senba T.,Matsumura K.,Nakajima Y.,Mizuno T.,Morinaga M.,Sasaki M.,Togashi T.,Oyama M.,Hata H.,Watanabe M.,Komatsu T.,Mizushima-Sugano J.,Satoh T.,Shirai Y.,Takahashi Y.,Nakagawa K.,Okumura K.,Nagase T.,Nomura N.,Kikuchi H.,Masuho Y.,Yamashita R.,Nakai K.,Yada T.,Nakamura Y.,Ohara O.,Isogai T.,Sugano S.
Complete sequencing and characterization of 21,243 full-length human cDNAs.
Nat. Genet.
36
40-45
2004
71092
Deloukas P.,Earthrowl M.E.,Grafham D.V.,Rubenfield M.,French L.,Steward C.A.,Sims S.K.,Jones M.C.,Searle S.,Scott C.,Howe K.,Hunt S.E.,Andrews T.D.,Gilbert J.G.R.,Swarbreck D.,Ashurst J.L.,Taylor A.,Battles J.,Bird C.P.,Ainscough R.,Almeida J.P.,Ashwell R.I.S.,Ambrose K.D.,Babbage A.K.,Bagguley C.L.,Bailey J.,Banerjee R.,Bates K.,Beasley H.,Bray-Allen S.,Brown A.J.,Brown J.Y.,Burford D.C.,Burrill W.,Burton J.,Cahill P.,Camire D.,Carter N.P.,Chapman J.C.,Clark S.Y.,Clarke G.,Clee C.M.,Clegg S.,Corby N.,Coulson A.,Dhami P.,Dutta I.,Dunn M.,Faulkner L.,Frankish A.,Frankland J.A.,Garner P.,Garnett J.,Gribble S.,Griffiths C.,Grocock R.,Gustafson E.,Hammond S.,Harley J.L.,Hart E.,Heath P.D.,Ho T.P.,Hopkins B.,Horne J.,Howden P.J.,Huckle E.,Hynds C.,Johnson C.,Johnson D.,Kana A.,Kay M.,Kimberley A.M.,Kershaw J.K.,Kokkinaki M.,Laird G.K.,Lawlor S.,Lee H.M.,Leongamornlert D.A.,Laird G.,Lloyd C.,Lloyd D.M.,Loveland J.,Lovell J.,McLaren S.,McLay K.E.,McMurray A.,Mashreghi-Mohammadi M.,Matthews L.,Milne S.,Nickerson T.,Nguyen M.,Overton-Larty E.,Palmer S.A.,Pearce A.V.,Peck A.I.,Pelan S.,Phillimore B.,Porter K.,Rice C.M.,Rogosin A.,Ross M.T.,Sarafidou T.,Sehra H.K.,Shownkeen R.,Skuce C.D.,Smith M.,Standring L.,Sycamore N.,Tester J.,Thorpe A.,Torcasso W.,Tracey A.,Tromans A.,Tsolas J.,Wall M.,Walsh J.,Wang H.,Weinstock K.,West A.P.,Willey D.L.,Whitehead S.L.,Wilming L.,Wray P.W.,Young L.,Chen Y.,Lovering R.C.,Moschonas N.K.,Siebert R.,Fechtel K.,Bentley D.,Durbin R.M.,Hubbard T.,Doucette-Stamm L.,Beck S.,Smith D.R.,Rogers J.
The DNA sequence and comparative analysis of human chromosome 10.
Nature
429
375-381
2004
71094
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
Genome Res.
14
2121-2127
2004
71095
Andersen J.S.,Wilkinson C.J.,Mayor T.,Mortensen P.,Nigg E.A.,Mann M.
Proteomic characterization of the human centrosome by protein correlation profiling.
Nature
426
570-574
2003
71096
Burkard T.R.,Planyavsky M.,Kaupe I.,Breitwieser F.P.,Buerckstuemmer T.,Bennett K.L.,Superti-Furga G.,Colinge J.
Initial characterization of the human central proteome.
BMC Syst. Biol.
5
17-17
2011
71097
Martinelli D.,Haeberle J.,Rubio V.,Giunta C.,Hausser I.,Carrozzo R.,Gougeard N.,Marco-Marin C.,Goffredo B.M.,Meschini M.C.,Bevivino E.,Boenzi S.,Colafati G.S.,Brancati F.,Baumgartner M.R.,Dionisi-Vici C.
Understanding pyrroline-5-carboxylate synthetase deficiency: clinical, molecular, functional, and expression studies, structure-based analysis, and novel therapy with arginine.
J. Inherit. Metab. Dis.
35
761-776
2012
71098
Wolthuis D.F.,van Asbeck E.,Mohamed M.,Gardeitchik T.,Lim-Melia E.R.,Wevers R.A.,Morava E.
Cutis laxa, fat pads and retinopathy due to ALDH18A1 mutation and review of the literature.
Eur. J. Paediatr. Neurol.
18
511-515
2014
71099
Bian Y.,Song C.,Cheng K.,Dong M.,Wang F.,Huang J.,Sun D.,Wang L.,Ye M.,Zou H.
An enzyme assisted RP-RPLC approach for in-depth analysis of human liver phosphoproteome.
J. Proteomics
96
253-262
2014
71100
Fischer-Zirnsak B.,Escande-Beillard N.,Ganesh J.,Tan Y.X.,Al Bughaili M.,Lin A.E.,Sahai I.,Bahena P.,Reichert S.L.,Loh A.,Wright G.D.,Liu J.,Rahikkala E.,Pivnick E.K.,Choudhri A.F.,Krueger U.,Zemojtel T.,van Ravenswaaij-Arts C.,Mostafavi R.,Stolte-Dijkstra I.,Symoens S.,Pajunen L.,Al-Gazali L.,Meierhofer D.,Robinson P.N.,Mundlos S.,Villarroel C.E.,Byers P.,Masri A.,Robertson S.P.,Schwarze U.,Callewaert B.,Reversade B.,Kornak U.
Recurrent de novo mutations affecting residue Arg138 of pyrroline-5-carboxylate synthase cause a progeroid form of autosomal-dominant cutis laxa.
Am. J. Hum. Genet.
97
483-492
2015
71101
Coutelier M.,Goizet C.,Durr A.,Habarou F.,Morais S.,Dionne-Laporte A.,Tao F.,Konop J.,Stoll M.,Charles P.,Jacoupy M.,Matusiak R.,Alonso I.,Tallaksen C.,Mairey M.,Kennerson M.,Gaussen M.,Schule R.,Janin M.,Morice-Picard F.,Durand C.M.,Depienne C.,Calvas P.,Coutinho P.,Saudubray J.M.,Rouleau G.,Brice A.,Nicholson G.,Darios F.,Loureiro J.L.,Zuchner S.,Ottolenghi C.,Mochel F.,Stevanin G.
Alteration of ornithine metabolism leads to dominant and recessive hereditary spastic paraplegia.
Brain
138
2191-2205
2015
71102
Vaca Jacome A.S.,Rabilloud T.,Schaeffer-Reiss C.,Rompais M.,Ayoub D.,Lane L.,Bairoch A.,Van Dorsselaer A.,Carapito C.
N-terminome analysis of the human mitochondrial proteome.
Proteomics
15
2519-2524
2015
71103
Panza E.,Escamilla-Honrubia J.M.,Marco-Marin C.,Gougeard N.,De Michele G.,Morra V.B.,Liguori R.,Salviati L.,Donati M.A.,Cusano R.,Pippucci T.,Ravazzolo R.,Nemeth A.H.,Smithson S.,Davies S.,Hurst J.A.,Bordo D.,Rubio V.,Seri M.
ALDH18A1 gene mutations cause dominant spastic paraplegia SPG9: loss of function effect and plausibility of a dominant negative mechanism.
Brain
139
0-0
2016
71105
Baumgartner M.R.,Hu C.A.,Almashanu S.,Steel G.,Obie C.,Aral B.,Rabier D.,Kamoun P.,Saudubray J.-M.,Valle D.
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.
Hum. Mol. Genet.
9
2853-2858
2000
71106
Bicknell L.S.,Pitt J.,Aftimos S.,Ramadas R.,Maw M.A.,Robertson S.P.
A missense mutation in ALDH18A1, encoding Delta1-pyrroline-5-carboxylate synthase (P5CS), causes an autosomal recessive neurocutaneous syndrome.
Eur. J. Hum. Genet.
16
1176-1186
2008
