WordNet

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

PrepTutorEJDIC

carbonの化学記号
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）』「2016/09/12 17:38:16」(JST)

wiki en

Methylmalonyl-CoA is the thioester consisting of coenzyme A linked to methylmalonic acid. It is an important intermediate in the biosynthesis of many organic compounds as well as in the process of carbon assimilation.^[1]

Biosynthesis and conversions

Methylmalonyl-CoA is formed from propionyl-CoA by propionyl-CoA carboxylase by help of biotin (vitamin B₇). It is converted into succinyl-CoA by methylmalonyl-CoA mutase, in a reaction that requires vitamin B₁₂ as a cofactor. In this way, it enters the Krebs cycle. The following diagram demonstrates the aforementioned reactions:^[2]

Propionyl CoA → Methylmalonyl CoA → Succinyl CoA

References

^ Tabita, F. R., "The hydroxypropionate pathway of CO₂ fixation: Fait accompli", Proceedings of the National Academy of Sciences 2009, vol. 106, 21015-21016. doi:10.1073/pnas.0912486107
^ Nelson, David L.; Cox, Michael M. (2005), Principles of Biochemistry (4th ed.), New York: W. H. Freeman, ISBN 0-7167-4339-6

This microbiology-related article is a stub. You can help Wikipedia by expanding it.

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. 脂質およびプリン代謝障害による代謝性ミオパチー metabolic myopathies caused by disorders of lipid and purine metabolism
2. 有機酸血症 organic acidemias
3. ビタミンB12および葉酸欠乏症の生理学 physiology of vitamin b12 and folate deficiency
4. 小児における溶血性尿毒症症候群の概要 overview of hemolytic uremic syndrome in children
5. ペルオキシソーム病 peroxisomal disorders

English Journal

Chronic administration of methylmalonate on young rats alters neuroinflammatory markers and spatial memory.

Ribeiro LR, Della-Pace ID, de Oliveira Ferreira AP, Funck VR, Pinton S, Bobinski F, de Oliveira CV, da Silva Fiorin F, Duarte MM, Furian AF, Oliveira MS, Nogueira CW, Dos Santos AR, Royes LF, Fighera MR.SourcePrograma de Pós-Graduação em Ciências Biológicas: Bioquímica Toxicológica, Universidade Federal de Santa Maria (UFSM), Brazil; Laboratório de Bioquímica do Exercício, Departamento de Métodos e Técnicas Desportivas, Centro de Educação Física e Desportos, UFSM, Brazil; Programa de Pós-graduação em Farmacologia, Centro de Ciências da Saúde, UFSM, Brazil.
Immunobiology.Immunobiology.2013 Sep;218(9):1175-83. doi: 10.1016/j.imbio.2013.04.008. Epub 2013 Apr 19.
The methylmalonic acidemia is an inborn error of metabolism (IEM) characterized by methylmalonic acid (MMA) accumulation in body fluids and tissues, causing neurological dysfunction, mitochondrial failure and oxidative stress. Although neurological evidence demonstrate that infection and/or inflamma
PMID 23726524

Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic acidemia.

Manoli I, Sysol JR, Li L, Houillier P, Garone C, Wang C, Zerfas PM, Cusmano-Ozog K, Young S, Trivedi NS, Cheng J, Sloan JL, Chandler RJ, Abu-Asab M, Tsokos M, Elkahloun AG, Rosen S, Enns GM, Berry GT, Hoffmann V, Dimauro S, Schnermann J, Venditti CP.SourceGenetics and Molecular Biology Branch, National Human Genome Research Institute, National Institutes of Health (NIH), Bethesda, MD 20892.
Proceedings of the National Academy of Sciences of the United States of America.Proc Natl Acad Sci U S A.2013 Jul 29. [Epub ahead of print]
Isolated methylmalonic acidemia (MMA), caused by deficiency of the mitochondrial enzyme methylmalonyl-CoA mutase (MUT), is often complicated by end stage renal disease that is resistant to conventional therapies, including liver transplantation. To establish a viable model of MMA renal disease, Mut
PMID 23898205

Japanese Journal

ビタミンB_<12>欠乏ラットの脳におけるOtx2遺伝子発現量の変動

勝島啓佑,上川篤志,河田哲典,前川昭男,小林謙一,田所忠弘,山本祐司
ビタミン 82(11), 595-599, 2008-11-25
… (B_<12>) is a coenzyme of methionine synthase (MS) and methylmalonyl-CoA mutase. …
NAID 110006990489

ビタミンB_<12>と精子形成(<特集>「ビタミン・ミネラルと生殖」[I])

山田和弘,河田哲典,前川昭男
ビタミン 82(1), 11-17, 2008-01-25
… is a cofactor for two enzymes, i.e. methionine synthase and methylmalonyl-CoA mutase. … According to earlier studies, methionine synthase, rather than methylmalonyl-CoA mutase, may be responsible for the testicular injury when animals are in B_<12>-deficiency. …
NAID 110006571393

Related Pictures

Metabolic interrelationships of methylmalonic acid, methylmalonyl CoA Chapter 16 : Oxidation of Fatty Acids Biochemistry of Methylmalonic Acidemia | Methylmalonic Acidemia Vit b12 Frontiers | Phylogenetic analysis of vitamin B12-related metabolism in Mycobacterium Oxidation and biosynthesis of fatty acids - ppt video online download Coa Methylmalonyl images Propionate metabolism - ppt download

★リンクテーブル★

リンク元	「ビオチン」「メチルマロニルCoA」
拡張検索	「methylmalonyl-CoA decarboxylase」「methylmalonyl-CoA racemase」
関連記事	「C」「CoA」「COA」「Co」

「ビオチン」

Methylmalonyl-CoA
Names
	IUPAC name (9R)-1-[(2R,3S,4R,5R)-5-(6-Amino-9H-purin-9-yl)-4-hydroxy-3-(phosphonooxy)tetrahydro-2-furanyl]-3,5,9-trihydroxy-8,8,20-trimethyl-10,14,19-trioxo-2,4,6-trioxa-18-thia-11,15-diaza-3,5-diphosphahenicosan-21-oic acid 3,5-dioxide (non-preferred name)
Identifiers
CAS Number	1264-45-5
ChEBI	CHEBI:16625
ChemSpider	110440
IUPHAR/BPS	5223
Jmol 3D model	Interactive image
PubChem	123909
	InChI InChI=1S/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12?,13-,16-,17-,18+,22-/m1/s1 Key: MZFOKIKEPGUZEN-FBMOWMAESA-N ; InChI=1/C25H40N7O19P3S/c1-12(23(37)38)24(39)55-7-6-27-14(33)4-5-28-21(36)18(35)25(2,3)9-48-54(45,46)51-53(43,44)47-8-13-17(50-52(40,41)42)16(34)22(49-13)32-11-31-15-19(26)29-10-30-20(15)32/h10-13,16-18,22,34-35H,4-9H2,1-3H3,(H,27,33)(H,28,36)(H,37,38)(H,43,44)(H,45,46)(H2,26,29,30)(H2,40,41,42)/t12?,13-,16-,17-,18+,22-/m1/s1 Key: MZFOKIKEPGUZEN-FBMOWMAEBZ ;
	SMILES CC(C(=O)O)C(=O)SCCNC(=O)CCNC(=O)[C@@H](C(C)(C)COP(=O)(O)OP(=O)(O)OC[C@@H]1[C@H]([C@H]([C@@H](O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O ;
Properties
Chemical formula	C25H40N7O19P3S
Molar mass	867.608 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