Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/12/03 23:13:03」(JST)

wiki en

Phosphoribosyl pyrophosphate (PRPP) is a pentosephosphate.

It is formed from ribose 5-phosphate by the enzyme ribose-phosphate diphosphokinase.

It plays a role in transferring phospho-ribose groups in several reactions:

Enzyme	Reactant	Product
adenine phosphoribosyltransferase	adenine	AMP
hypoxanthine-guanine phosphoribosyltransferase	guanine	GMP
hypoxanthine-guanine phosphoribosyltransferase	hypoxanthine	IMP
orotate phosphoribosyltransferase	orotate	OMP
uracil phosphoribosyltransferase	uracil	UMP

In de novo generation of purines, the enzyme amidophosphoribosyltransferase acts upon PRPP to create phosphoribosylamine.

Increased PRPP

Increased levels of PRPP is characterized by the overproduction and accumulation of uric acid leading to hyperuricemia and hyperuricosuria. It is one of the causes of gout.

Increased levels of PRPP are present in Lesch-Nyhan Syndrome. Decreased levels of hypoxanthine guanine phosphoribosyl transferase (HGPRT) causes this accumulation, as PRPP is a substrate used by HGPRT during purine salvage.

English Journal

Mutations in PRPS1 causing syndromic or nonsyndromic hearing impairment: intrafamilial phenotypic variation complicates genetic counseling.

Gandía M1, Fernández-Toral J2, Solanellas J3, Domínguez-Ruiz M1, Gómez-Rosas E1, Del Castillo FJ1, Villamar M1, Moreno-Pelayo MA1, Del Castillo I1.
Pediatric research.Pediatr Res.2015 Mar 18. doi: 10.1038/pr.2015.56. [Epub ahead of print]
BACKGROUND: PRPS1 encodes isoform I of phosphoribosylpyrophosphate synthetase (PRS-I), a key enzyme in nucleotide biosynthesis. Different missense mutations in PRPS1 cause a variety of disorders that include PRS-I superactivity, nonsyndromic sensorineural hearing impairment, Charcot-Marie-Tooth dise
PMID 25785835

Purinosome formation as a function of the cell cycle.

Chan CY1, Zhao H2, Pugh RJ2, Pedley AM2, French J3, Jones SA4, Zhuang X5, Jinnah H6, Huang TJ1, Benkovic SJ7.
Proceedings of the National Academy of Sciences of the United States of America.Proc Natl Acad Sci U S A.2015 Feb 3;112(5):1368-73. doi: 10.1073/pnas.1423009112. Epub 2015 Jan 20.
The de novo purine biosynthetic pathway relies on six enzymes to catalyze the conversion of phosphoribosylpyrophosphate to inosine 5'-monophosphate. Under purine-depleted conditions, these enzymes form a multienzyme complex known as the purinosome. Previous studies have revealed the spatial organiza
PMID 25605889

Novel C12orf65 mutations in patients with axonal neuropathy and optic atrophy.

Tucci A1, Liu YT, Preza E, Pitceathly RD, Chalasani A, Plagnol V, Land JM, Trabzuni D, Ryten M; UKBEC, Jaunmuktane Z, Reilly MM, Brandner S, Hargreaves I, Hardy J, Singleton AB, Abramov AY, Houlden H.
Journal of neurology, neurosurgery, and psychiatry.J Neurol Neurosurg Psychiatry.2014 May;85(5):486-92. doi: 10.1136/jnnp-2013-306387. Epub 2013 Nov 6.
OBJECTIVE: Charcot-Marie Tooth disease (CMT) forms a clinically and genetically heterogeneous group of disorders. Although a number of disease genes have been identified for CMT, the gene discovery for some complex form of CMT has lagged behind. The association of neuropathy and optic atrophy (also
PMID 24198383

Japanese Journal

Effect of Amplification of Desensitized purF and prs on Inosine Accumulation in Escherichia coli(MICROBIAL PHYSIOLOGY AND BIOTECHNOLOGY)

Journal of bioscience and bioengineering 103(3), 255-261, 2007-03-25
NAID 110006271247

Effect of Amplification of Desensitized purF and prs on Inosine Accumulation in Escherichia coli

Journal of Bioscience and Bioengineering 103(3), 255-261, 2007
NAID 130004144753

Amidophosphoribosyltransferase(ATase) と Phosphoribosylpyrophosphate (PRPP) 合成酵素遺伝子多型と産生過剰型痛風の関連解析

痛風と核酸代謝 = Gout and nucleic acid metabolism 30(1), 79, 2006-07-01
NAID 10017657087

Related Pictures

Immunohistochemistry (Formalin/PFA-fixed diphosphate,phosphoribosylpyrophosphate Thumb PRPP , phosphoribosyl File:Phosphoribosyl pyrophosphate.svg Phosphoribosyl Pyrophosphate (PRPP) PRPP, PHOSPHORIBOSYL PYROPHOSPHATE Phosphoribosyl-1-pyrophosphate

★リンクテーブル★

リンク元	「6-メルカプトプリン」「5-ホスホリボシル1α-二リン酸」「PRPP synthetase superactivity」「PRS superactivity」

「6-メルカプトプリン」

Phosphoribosyl pyrophosphate
Names
	Other names 5-phospho-alpha-D-ribose 1-diphosphate
Identifiers
CAS Number	7540-64-9
ChEBI	CHEBI:17111
ChemSpider	7062
DrugBank	DB01632
	InChI InChI=1S/C5H13O14P3/c6-3-2(1-16-20(8,9)10)17-5(4(3)7)18-22(14,15)19-21(11,12)13/h2-7H,1H2,(H,14,15)(H2,8,9,10)(H2,11,12,13)/t2-,3-,4-,5-/m1/s1 Key: PQGCEDQWHSBAJP-TXICZTDVSA-N ; InChI=1/C5H13O14P3/c6-3-2(1-16-20(8,9)10)17-5(4(3)7)18-22(14,15)19-21(11,12)13/h2-7H,1H2,(H,14,15)(H2,8,9,10)(H2,11,12,13)/t2-,3-,4-,5-/m1/s1 Key: PQGCEDQWHSBAJP-TXICZTDVBW ;
Jmol interactive 3D	Image
MeSH	Phosphoribosyl+pyrophosphate
PubChem	7339
	SMILES O=P(O[C@H]1O[C@@H]([C@@H](O)[C@H]1O)COP(=O)(O)O)(O)OP(=O)(O)O ;
Properties
Chemical formula	C5H13O14P3
Molar mass	390.07 g/mol
	Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
	verify (what is ?)
	Infobox references

　　[★]

英: 6-mercaptopurine, mercaptopunne
ラ: 6-mercaptopurinum
同: 6-MP
商: ロイケリン
関: アザチオプリン、制癌薬

GOO. 134-138,1347f
プリン代謝拮抗薬
抗悪性腫瘍薬

白血病治療薬

プリンの6位にSが付加された物質。

代謝

投与された6-MPはPPRP(phosphoribosylpyrophosphateにより6-thioinosine 5'-monophosphate(TIMP)となる
6-thioinosine 5'-monophosphateはいくつかの代謝を経て6-thioguanosine 5'-monophosphate(TGN)となる

作用機序

TIMPがプリン塩基の生合成を阻害する

本剤は、細胞増殖に重要な意義をもつ核酸の生合成を阻害する。メルカプトプリンは細胞内でinosinic acidのチオ同族体 thioinosinic acid（TIMP）に変換し、このTIMPは主としてinosinic acid からのadenylosuccinic acid 及びxanthylic acidへの転換を阻害し、adenine, guanineribonucleotideの生合成を阻害するとされている