succinyl-CoA

サクシニル補酵素A、サクシニルCoA

関

succinyl-coenzyme A

WordNet

1. the 3rd letter of the Roman alphabet (同)c
2. (music) the keynote of the scale of C major
3. a general-purpose programing language closely associated with the UNIX operating system

PrepTutorEJDIC

1. carbonの化学記号
2. cobaltの化学記号

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2012/10/18 01:48:11」(JST)

wiki en

[Wiki en表示]

Succinyl-CoA
Identifiers
CAS number	604-98-8
PubChem	92133
ChemSpider	83179 Y
MeSH	succinyl-coenzyme+A
ChEBI	CHEBI:15380 Y
Jmol-3D images	Image 1
SMILES O=C(O)CCC(=O)SCCNC(=O)CCNC(=O)[C@H](O)C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@H]3O[C@@H](n2cnc1c(ncnc12)N)[C@H](O)[C@@H]3OP(=O)(O)O
InChI InChI=1S/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20+,24-/m1/s1 Y Key: VNOYUJKHFWYWIR-ITIYDSSPSA-N Y InChI=1/C25H40N7O19P3S/c1-25(2,20(38)23(39)28-6-5-14(33)27-7-8-55-16(36)4-3-15(34)35)10-48-54(45,46)51-53(43,44)47-9-13-19(50-52(40,41)42)18(37)24(49-13)32-12-31-17-21(26)29-11-30-22(17)32/h11-13,18-20,24,37-38H,3-10H2,1-2H3,(H,27,33)(H,28,39)(H,34,35)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t13-,18-,19-,20+,24-/m1/s1 Key: VNOYUJKHFWYWIR-ITIYDSSPBJ
Properties
Molecular formula	C25H40N7O19P3S
Molar mass	867.608
Y (verify) (what is: Y/N?) Except where noted otherwise, data are given for materials in their standard state (at 25 °C, 100 kPa)
Infobox references

Succinyl-Coenzyme A, abbreviated as Succinyl-CoA or SucCoA, is a combination of succinic acid and coenzyme A.

Source

It is an important intermediate in the citric acid cycle, where it is synthesized from α-Ketoglutarate by α-ketoglutarate dehydrogenase through decarboxylation. During the process, coenzyme A is added.

It is also synthesized from propionyl CoA, the odd-numbered fatty acid, which cannot undergo beta-oxidation. Propionyl-CoA is carboxylated to D-methylmalonyl-CoA, isomerized to L-methylmalonyl-CoA, and rearranged to yield succinyl-CoA via a vitamin B₁₂-dependent enzyme. Succinyl-CoA is an intermediate of the citric acid cycle and can be readily incorporated there.

Fate

It is converted into succinate through the hydrolytic release of coenzyme A by succinyl-CoA synthetase (succinate thiokinase).

Another fate of succinyl-CoA is porphyrin synthesis, where succinyl-CoA and glycine are combined by ALA synthase to form δ-aminolevulinic acid (dALA).

Formation

Succinyl CoA can be formed from methylmalonyl CoA through the utilization of deoxyadenosyl-B₁₂ (deoxyadenosylcobalamin) by the enzyme methylmalonyl-CoA mutase. This reaction, which requires vitamin B₁₂ as a cofactor, is important in the catabolism of some branched-chain amino acids as well as odd-chain fatty acids.

Interactive pathway map

Click on genes, proteins and metabolites below to link to respective articles.

[[File:

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

[[

]]

|{{{bSize}}}px]]

Citric_acid_cycle edit

References

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

• 1. ミトコンドリアミオパシー：臨床的特徴および診断 mitochondrial myopathies clinical features and diagnosis

English Journal

• Localization of SUCLA2 and SUCLG2 subunits of succinyl CoA ligase within the cerebral cortex suggests the absence of matrix substrate-level phosphorylation in glial cells of the human brain.

• Dobolyi A1, Bagó AG, Gál A, Molnár MJ, Palkovits M, Adam-Vizi V, Chinopoulos C.
• Journal of bioenergetics and biomembranes.J Bioenerg Biomembr.2015 Apr;47(1-2):33-41. doi: 10.1007/s10863-014-9586-4. Epub 2014 Nov 5.
• We have recently shown that the ATP-forming SUCLA2 subunit of succinyl-CoA ligase, an enzyme of the citric acid cycle, is exclusively expressed in neurons of the human cerebral cortex; GFAP- and S100-positive astroglial cells did not exhibit immunohistoreactivity or in situ hybridization reactivity
• PMID 25370487

• Mitochondrial encephalomyopathy and retinoblastoma explained by compound heterozygosity of SUCLA2 point mutation and 13q14 deletion.

• Matilainen S1, Isohanni P2, Euro L1, Lönnqvist T3, Pihko H3, Kivelä T4, Knuutila S5, Suomalainen A6.
• European journal of human genetics : EJHG.Eur J Hum Genet.2015 Mar;23(3):325-30. doi: 10.1038/ejhg.2014.128. Epub 2014 Jul 2.
• Mutations in SUCLA2, encoding the ß-subunit of succinyl-CoA synthetase of Krebs cycle, are one cause of mitochondrial DNA depletion syndrome. Patients have been reported to have severe progressive childhood-onset encephalomyopathy, and methylmalonic aciduria, often leading to death in childhood. We
• PMID 24986829

• Mitochondrial engineering of the TCA cycle for fumarate production.

• Chen X1, Dong X1, Wang Y1, Zhao Z1, Liu L2.
• Metabolic engineering.Metab Eng.2015 Feb 20. pii: S1096-7176(15)00013-0. doi: 10.1016/j.ymben.2015.02.002. [Epub ahead of print]
• Microbial fumarate production from renewable feedstock is a promising and sustainable alternative to petroleum-based chemical synthesis. Here, mitochondrial engineering was used to construct the oxidative pathway for fumarate production starting from the TCA cycle intermediate α-ketoglutarate in Ca
• PMID 25708514

Japanese Journal

• RpoS and oxidative stress conditions regulate succinyl-CoA : 3-ketoacid-coenzyme A transferase (SCOT) expression in Burkholderia pseudomallei

• Chutoam Palatip,Charoensawan Varodom,Wongtrakoongate Patompon [他]
• Microbiology and immunology 57(9), 605-615, 2013-09
• NAID 40019798437

• Archaeal Aldehyde Dehydrogenase ST0064 from Sulfolobus tokodaii, a Paralog of Non-Phosphorylating Glyceraldehyde-3-phosphate Dehydrogenase, Is a Succinate Semialdehyde Dehydrogenase

• ITO Fumiaki,CHISHIKI Hidehiro,FUSHINOBU Shinya [他],WAKAGI Takayoshi
• Bioscience, Biotechnology, and Biochemistry 77(6), 1344-1348, 2013
• … Activity toward GAP was lower (Km of 4.6 mM and kcat of 4.77 s−1), and previously predicted succinyl-CoA reductase activity was not detected, suggesting that the enzyme functions practically as succinate semialdehyde dehydrogenase (SSADH). …
• NAID 130003361114

• A Comparative Analysis of the Molecular Characteristics of the Arabidopsis CoA Pyrophosphohydrolases AtNUDX11, 15, and 15a

• ITO Daisuke,YOSHIMURA Kazuya,ISHIKAWA Kazuya [他],OGAWA Takahisa,MARUTA Takanori,SHIGEOKA Shigeru
• Bioscience, biotechnology, and biochemistry 76(1), 139-147, 2012-01-23
• … Coenzyme A (CoA) is an essential, ubiquitous cofactor in all biological systems, where it acts as the major acyl group carrier in various central metabolic reactions. … Although much is known about CoA biosynthesis, it is unclear how the CoA pool is regulated the various cellular compartments. … It has been found that the nucleoside diphosphates linked to some moiety X (Nudix) hydrolases, AtNUDX11 and 15, have pyrophosphohydrolase activity toward CoA and its derivatives. …
• NAID 10030398798

Related Links

• succinyl-CoA - definition of succinyl-CoA by Medical dictionary

succinyl-CoA a high energy intermediate metabolite formed in the Krebs cycle by the oxidation of α-ketoglutaric acid. suc·ci·nyl-co·en·zyme A (sŭk'si-nil-kō-en'zīm), The condensation product of succinic acid and CoA; one of the ...

• succinyl CoA - definition of succinyl CoA by Medical dictionary

succinyl CoA /suc·ci·nyl CoA/ (suk´sĭ-nil ko-a´) a high-energy intermediate formed in the tricarboxylic acid cycle from α-ketoglutaric acid; it is also a ... Disclaimer All content on this website, including dictionary, thesaurus, literature ...

Related Pictures

---

★リンクテーブル★

リンク元	「ヘム」「プロピオニルCoA」「スクシニルCoA」「succinyl-coenzyme A」
拡張検索	「succinyl-CoA-acetoacetate-CoA transferase」「succinyl-CoA synthetase」
関連記事	「C」「CoA」「COA」「Co」

「ヘム」

　　[★]

英

heme

同

プロトヘム protoheme、フェロプロトポルフィリンIX ferroprotoporphyrin IX

関

ヘマチン hematin

• ポルフィリンと鉄イオンの錯体

生合成

• →ポルフィリン

反応場所							酵素	補酵素	酵素阻害物質	酵素の障害による疾患
ミトコンドリア	サクシニルCoA succinyl-CoA	グリシン glycine		5-アミノレブリン酸 aminolevulinic acid aminolevulinate ALA	CoA	CO2	5-アミノレブリン酸シンターゼ ALA synthase	ビタミンB6
細胞質	5-アミノレブリン酸 aminolevulinic acid aminolevulinate ALA	←2分子		ポルホビリノゲン porphobilinogen PBG	2H2O		ALA dehydratase		Pb
細胞質	ポルホビリノゲン porphobilinogen PBG			ヒドロキシメチルビラン	4NH3		ウロポルフィリゲノンIシンターゼ uroporphyrinogen I synthase			急性間欠性ポルフィリン症 acute intermittent porphyria
細胞質	ヒドロキシメチルビラン			ウロポルフィリノゲンIII			ウロポルフィリゲノンIIIシンターゼ uroporphyrinogen III synthase
細胞質	ウロポルフィリノゲンIII			コプロポルフィリノゲンIII	4CO2		ウロポルフィリノゲンデカルボキシラーゼ			晩発性皮膚ポルフィリン症 porphyria cutanea tarda
ミトコンドリア	コプロポルフィリノゲンIII			プロトポルフィリノゲンIX	2CO2		コプロポルフィリノゲンオキシダーゼ
ミトコンドリア	プロトポルフィリノゲンIX			プロトポルフィリンIX			プロトポルフィリノゲンオキシダーゼ
ミトコンドリア	プロトポルフィリンIX	Fe2+		ヘム			フェロケラターゼ		Pb

生合成の場所

• 哺乳類細胞では、赤血球以外は何処でも行われる(ミトコンドリアを有するなら)
• 実際には、赤血球前駆細胞(erythroid precursor cell)で85%行われており、残りは肝細胞で行われている。

「プロピオニルCoA」

　　[★]

英

propionyl CoA

関

[[]]

• プロピオニルCoA propionyl CoA

• プロピオニルCoAカルボキシラーゼ propionyl-CoA carboxylase：ビオチン

• (S)-メチルマロニルCoA (S)-methylmaronyl-CoA

• メチルマロニルCoAエピメラーゼ methylmalonyl-CoA epimerase

• (R)-メチルマロニルCoA (R)-methylmaronyl-CoA

• メチルマロニルCoAムターゼ methylmalonyl-CoA mutase：ビタミンB12

• スクシニルCoA succinyl-CoA

同

propiony卜CoA

「スクシニルCoA」

　　[★]

英

succinyl-CoA succinyl coenzyme A

関

コハク酸 succinate

• コハク酸 succinate

HOOC-CH2-CH2-COOH

• スクシニルCoA

HOOC-CH2-CO-S-CoA

「succinyl-coenzyme A」

　　[★]

• サクシニル補酵素A、サクシニルコエンザイムA、サクシニルCoA

関

succinyl-CoA

「succinyl-CoA-acetoacetate-CoA transferase」

　　[★]

同

thiophorase, succinyl CoA:acetoacetate CoA-transferase, succinyl-CoA acetoacetate CoA transferase

「succinyl-CoA synthetase」

　　[★] スクシニルCoAシンテターゼ

「C」

　　[★]

• 炭素 carbon
• シトシン cytosine
• システイン cysteine
• シチジン
• セルシウス度 摂氏 degrees Celsius degree C
• 産婦人科領域で「子宮外頚 子宮外頚部 exocervix」を表現するための略語

　 　 　 　

「CoA」

　　[★]

• 大動脈縮窄症 coactation of the aorta
• 補酵素A coenzyme A

「COA」

　　[★] 補酵素A coenzyme A CoA

「Co」

　　[★] コバルト cobalt