biopterin

ビオプテリン

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/01/31 10:26:51」(JST)

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Biopterin

Names
IUPAC name 2-Amino-6-(1,2-dihydroxypropyl)-1H-pteridin-4-one
Identifiers
CAS Number	22150-76-1 Y
3D model (Jmol)	Interactive image Interactive image
ChemSpider	392795 Y
ECHA InfoCard	100.040.719
KEGG	C06313 Y
MeSH	Biopterin
PubChem	445040
InChI InChI=1S/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h2-3,6,15-16H,1H3,(H3,10,11,13,14,17)/t3-,6-/m0/s1 Y Key: LHQIJBMDNUYRAM-DZSWIPIPSA-N Y InChI=1/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h2-3,6,15-16H,1H3,(H3,10,11,13,14,17) Key: LHQIJBMDNUYRAM-UHFFFAOYAC InChI=1/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h2-3,6,15-16H,1H3,(H3,10,11,13,14,17)/t3-,6-/m0/s1 Key: LHQIJBMDNUYRAM-DZSWIPIPBD
SMILES O=C2\N=C(/Nc1ncc(nc12)C(O)C(O)C)N O=C2\N=C(/Nc1ncc(nc12)[C@@H](O)[C@@H](O)C)N
Properties
Chemical formula	C9H11N5O3
Molar mass	237.216 g/mol
Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 kPa).
Y verify (what is YN ?)
Infobox references

Biopterins are pterin derivatives which function as endogenous enzyme cofactors in many species of animals and in some bacteria and fungi. Biopterins act as cofactors for aromatic amino acid hydroxylases (AAAH), which are involved in the synthesis a number of neurotransmitters including dopamine, norepinephrine, epinepherine, and serotonin, along with several trace amines; nitric oxide synthesis also utilizes biopterin derivatives as cofactors. In humans, tetrahydrobiopterin is the endogenous cofactor for AAAH enzymes.

Compounds

Biopterin compounds found within the body include both BH4 and BH2.

Synthesis

Biopterin synthesis occurs through two principal pathways; the de novo pathway involves three enzymatic steps and proceeds from GTP, while the salvage pathway converts sepiapterin to biopterin. BH4 is the principal active cofactor, and a recycling pathway converts BH2 to BH4.

Biopterin disorders

Main articles: Tetrahydrobiopterin deficiency and Tetrahydrobiopterin-deficient hyperphenylalaninemia

A number of disorders of biopterin regulation exist.

Single-gene defects affecting the gene GCH1 block the first step in biopterin synthesis, and lead to dopamine-responsive dystonia, also known as Segawa's syndrome. This is due to the role of BH4 in synthesising neurotransmitters, including Dopamine, and is treated with supplementation with levodopa, which does not require BH4 for conversion to dopamine. GCH1 defects are autosomal dominant, meaning that only one defective gene copy is required for the condition to occur. Mouse gene knockout models that block biopterin synthesis completely die shortly after birth due to their inability to produce catecholamines and neurotransmitters. Biopterin synthesis disorders are also a cause of hyperphenylalaninemia; phenylalanine metabolism requires BH4 as a cofactor.

References

External links

• Neurological aspects of biopterin metabolism

WiseGeek. (2012). What is biopterin?. Retrieved from http://www.wisegeek.com/what-is-biopterin.htm

UpToDate Contents

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• 1. フェニルケトン尿症の概要 overview of phenylketonuria

English Journal

• Rapid clearance of supplemented tetrahydrobiopterin is driven by high-capacity transporters in the kidney.

• Ohashi A, Suetake Y, Saeki Y, Harada T, Aizawa S, Hasegawa H.SourceDivision of Anatomical Science, Department of Functional Morphology, Nihon University School of Medicine, Itabashi, Tokyo 173-8610, Japan.
• Molecular genetics and metabolism.Mol Genet Metab.2012 Apr;105(4):575-81. Epub 2012 Jan 21.
• Tetrahydrobiopterin (BH(4)) is an essential cofactor of aromatic amino acid hydroxylases and NO synthase. Supplementation of BH(4) potentially targets cardiovascular dysfunction as well as inherited BH(4) deficiencies and BH(4)-responsive phenylketonuria. However, the high cost/effect ratio of the r
• PMID 22318121

• Systemic and vascular oxidation limits the efficacy of oral tetrahydrobiopterin treatment in patients with coronary artery disease.

• Cunnington C, Van Assche T, Shirodaria C, Kylintireas I, Lindsay AC, Lee JM, Antoniades C, Margaritis M, Lee R, Cerrato R, Crabtree MJ, Francis JM, Sayeed R, Ratnatunga C, Pillai R, Choudhury RP, Neubauer S, Channon KM.SourceDepartment of Cardiovascular Medicine, University of Oxford, John Radcliffe Hospital, Oxford, UK.
• Circulation.Circulation.2012 Mar 20;125(11):1356-66. Epub 2012 Feb 7.
• BACKGROUND: The endothelial nitric oxide synthase cofactor tetrahydrobiopterin (BH4) plays a pivotal role in maintaining endothelial function in experimental vascular disease models and in humans. Augmentation of endogenous BH4 levels by oral BH4 treatment has been proposed as a potential therapeuti
• PMID 22315282

Japanese Journal

• ビオプテリン測定の現状と課題(<特集>ビタミン測定法の現状と課題[V])

• 一瀬 宏,小柴 梢子,本田 俊一郎,徳岡 宏文
• ビタミン 85(10), 547-551, 2011-10-25
• … Here, we introduce the post-column oxidation method developed by Tani and Ohno for discriminative quantitation of the reduced forms of pteridines, including BH4, BH2, and biopterin, in biological samples. …
• NAID 110008762042

• テトラヒドロビオプテリンの細胞膜輸送体活性の測定 : アフリカツメガエル卵母細胞による輸送体遺伝子発現系の応用(<特集>ビタミン測定法の現状と課題[IV])

• 大橋 晶子,菅原 ゆうこ,真々田 薫,安西 尚彦,相澤 信,長谷川 宏幸
• ビタミン 85(9), 485-491, 2011-09-25
• Supplementation of tetrahydrobiopterin (BH_4), an essential cofactor in nitric oxide and monoamine production, is used in treating a systemic deficiency of BH_4 despite of high cost medication due to …
• NAID 110008723139

Related Links

• ビオプテリン - 辞書すべて - goo辞書

ビオプテリン(biopterin)は、体内で合成される補酵素の一つ。テトラヒドロビオプテリンの酸化分解産物である。 専門用語集 の検索結果 家庭の医学の検索結果 … 1件 ビオプテリン異常症 「ビオプテリン」の使用例を検索する "Let's get up ...

• ビオプテリン | ローヤルゼリーはどれが良い？ローヤルゼリー配合 ...

ローヤルゼリー配合のビオプテリンの効果、効能について ... 美容、健康に効果のあると言われるローヤルゼリー。ローヤルゼリーの効果、効能を調査。ローヤルゼリー配合製品のそれぞれの特徴、メリット、効果をチェック。

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★リンクテーブル★

リンク元	「カテコールアミン」
拡張検索	「tetrahydrobiopterin-synthesizing enzyme」「tetrahydrobiopterin」「5,6,7,8-tetrahydrobiopterin」「dihydrobiopterin」

「カテコールアミン」

　　[★]

英

catecholamine catecholamines CA

同

カテコラミン

関

副腎髄質ホルモン

• カテコールでかつアミンを持つ構造

Wikipedia

• ドーパミン、ノルアドレナリン、アドレナリン

生合成

チロシン tyrosine
 (フェニル基の3位にOHを導入) ：律速酵素
 -チロシン 3-モノオキシゲナーゼ tyrosine 3-monooxygenase, チロシン水酸化酵素 tyrosine hydroxylase
 ←{H4・biopterin}
 →{H2・biopterin}
L-ドーパ L-dopa
 (アミノ酸から脱炭酸)
 -芳香族アミノ酸デカルボキシラーゼ dopa decarboxylase + {ピリドキサル5-リン酸(VB6)}
 →CO2
ドパミン dopamine
 (β位の炭素にOHを導入)
 -ドーパミン β-モノオキシゲナーゼ dopamine β-monoxygenase, dopamine β-odidase
 ←O2 + ビタミンC(アスコルビン酸)
 →H2O + デヒドロアスコルビン酸
ノルアドレナリン norepinephrine
 (アミノ基にメチル基を転移)
 -フェニルエタノールアミン N-メチルトランスフェラーゼ phenylethanolamine N-methyl transferase
 ←S-アデノシルメチオニン S-adenosylmethionine
 →S-アデノシルホモシステイン S-adenosylhemocysteine
アドレナリン epinephrine

分解

• ドパミン、ノルアドレナリン、アドレナリンはいずれも以下の酵素により分解される

• MAO monoamine oxidase
• COMT cathechol O-methyltransferase

分解産物

ドパミン dopamine	－－－→	ジヒドロキシフェニル酢酸 3,4-dihydroxyphenylacetic acid DOPAC	－－－→	ホモバニリン酸 homovanillic acid HVA
｜ ｜	MAO COMT
｜ ｜ ↓
ノルエピネフリン norepinephrine	－－－→	バニリルマンデリン酸 vanillylmandelic acid VMA 　　　　　　　・ メトキシヒドロキシフェニルグリコール methoxyhydroxyphenylglycol MHPG
｜ ｜ ↓	MAO COMT
エピネフリン epinephrine	－－－→
	MAO COMT

薬理作用

昇圧作用

• (ドパミン、ドブタミン)アシドーシス存在下で効力低下

臨床検査

• 褐色細胞腫、神経芽腫の診断に用いられる。

基準値

カテコールアミン３分画(血漿)(BML)

• アドレナリン：≦0.10 ng/mL
• ノルアドレナリン：0.10～0.50 ng/mL
• ドパミン：≦0.03 ng/mL

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「tetrahydrobiopterin-synthesizing enzyme」

　　[★] テトラヒドロビオプテリン合成酵素

「tetrahydrobiopterin」

　　[★] テトラヒドロビオプテリン

「5,6,7,8-tetrahydrobiopterin」

　　[★]

5

関

tetrahydrobiopterin

「dihydrobiopterin」

　　[★]

ジヒドロビオプテリン