Metilmalonil-CoA

 
Metilmalonil-CoA
General
Otros nombres Metilmalonil coenzima A
Fórmula estructural Imagen de la estructura
Fórmula molecular C25H40N7O19P3S
Identificadores
Número CAS 317-66-81264-45-5[1]
ChEBI 16625
ChemSpider 110440
PubChem 123909
KEGG C02557
Propiedades físicas
Masa molar 8676 g/mol
Valores en el SI y en condiciones estándar
(25 y 1 atm), salvo que se indique lo contrario.

El metilmalonil-CoA es el tioéster de la coenzima A del ácido metilmalónico. Es un intermedio importante en la biosíntesis de muchos compuestos orgánicos, así como en el proceso de asimilación de carbono.[2]

El metilmalonil-CoA se produce como un intermediario en la degradación de los ácidos grasos con un número impar de átomos de carbono[3]​ y algunos aminoácidos (tales como isoleucina, valina, metionina y treonina).[4]​ El producto formado durante la degradación de metilmalonil-CoA es el succinil-coA producido en el metabolismo y en el ciclo del ácido cítrico, y la reacción con guanosín difosfato (GDP) y fosfato libre es catalizada por la succinil-CoA sintetasa, y suministra el análogo al ATP, el guanosin trifosfato (GTP).

Biosíntesis

Los dos enantiómeros, L y D (o R y S), del metilmalonil-CoA intervienen en la degradación de los lípidos a través de la secuencia de reacciones siguiente :

El siguiente diagrama muestra las reacciones antes mencionadas:

Propionil-CoA → Metilmalonil CoA → Succinil-coA

También está implicado en la degradación de ciertos aminoácidos tales como isoleucina, metionina, treonina y valina.

Isómeros

Estructura del L-metilmalonil-CoA (a la izquierda) y del D-metilmalonil-CoA (a la derecha). Si usamos las reglas de Cahn-Ingold-Prelog (CIP), tenemos que el enantiómero L se llamaría R, y el D sería el S.

Enfermedades relacionadas

En la enfermedad metabólica aciduria malónica y metilmalónica combinada (CMAMMA) debida a la deficiencia de ACSF3, se reduce la metilmalonil-CoA sintetasa, que convierte el ácido metilmalónico tóxico en metilmalonil-CoA y lo suministra así al ciclo de Krebs.[5][6]​ El resultado es una acumulación de ácido metilmalónico.

Bibliografía

  1. Número CAS
  2. Tabita, F. Robert (2009). «The hydroxypropionate pathway of CO2 fixation: Fait accompli». PNAS 106 (50): 21015-21016. doi:10.1073/pnas.0912486107. 
  3. a b H. Robert Horton, Laurence A. Moran, K. Gray Scrimgeour, J. David Rawn, Marc D. Perry: Biochemie. 4. Auflage, Pearson Education, 2008, ISBN 978-3-8273-7312-0, S. 674–675
  4. R. Witkowski, O. Prokop, E. Ullrich: Lexikon der Syndrome und Fehlbildungen: Ursachen, Genetik, Risiken. 7. Auflage, Springer, 2003, ISBN 978-3-540-44305-6, S. 818
  5. Gabriel, Marie Cosette; Rice, Stephanie M.; Sloan, Jennifer L.; Mossayebi, Matthew H.; Venditti, Charles P.; Al‐Kouatly, Huda B. (2021-04). «Considerations of expanded carrier screening: Lessons learned from combined malonic and methylmalonic aciduria». Molecular Genetics & Genomic Medicine (en inglés) 9 (4). ISSN 2324-9269. PMC 8123733. PMID 33625768. doi:10.1002/mgg3.1621. 
  6. Bowman, Caitlyn E.; Wolfgang, Michael J. (2019-01). «Role of the malonyl-CoA synthetase ACSF3 in mitochondrial metabolism». Advances in Biological Regulation (en inglés) 71: 34-40. PMC 6347522. PMID 30201289. doi:10.1016/j.jbior.2018.09.002. 

Read other articles:

Pagalungan Munisipalitas di Filipina Tempat Negara berdaulatFilipinaIsland group of the Philippines (en) kepulauan MindanauRegion of the Philippines (en) BangsamoroProvinsi di FilipinaMaguindanao del Sur (en) NegaraFilipina PendudukTotal46.277  (2020 )Tempat tinggal7.831  (2020 )Bahasa resmibahasa Magindanau dan Tagalog GeografiLuas wilayah898,76 km² [convert: unit tak dikenal]Ketinggian17 m Berbatasan denganPikit Informasi tambahanKode pos9610 Kode telepon64 Lain-lainSitus we...

 

Masjid Agung Ciamisمسجد الجامع چيءميسMasjid Agung Ciamis dilihat dari arah Tenggara, 2012AgamaAfiliasi agamaIslamProvinsi Jawa BaratLokasiLokasiJalan Jenderal Sudirman №3, Ciamis, IndonesiaKoordinat7°19′35.972″S 108°21′3.740″E / 7.32665889°S 108.35103889°E / -7.32665889; 108.35103889Koordinat: 7°19′35.972″S 108°21′3.740″E / 7.32665889°S 108.35103889°E / -7.32665889; 108.35103889{{#coordinates:}}: ti...

 

1941 brief attempt at invading Thailand by Imperial Japan Japanese invasion of ThailandPart of the Pacific War of World War IIMap of the Japanese invasion of Thailand, December 8, 1941Date8 December 1941(5 hours)LocationThailandResult Ceasefire Thai alliance with Japan Thailand declares war on the Allied Powers.Belligerents Thailand JapanCommanders and leaders Plaek Pibulsonggram Shōjirō Iida Tomoyuki Yamashita Nobutake KondoUnits involved Royal Thai Army Royal Thai Air Force Royal Thai Nav...

Indian politician (1914–2009) This article needs additional citations for verification. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed.Find sources: Ranbir Singh Hooda – news · newspapers · books · scholar · JSTOR (October 2021) (Learn how and when to remove this template message) ChaudharyRanbir Singh HoodaPersonal detailsBorn26 November 1914[1]Sanghi, Punjab Provinc...

 

بطولة العالم للمصارعةمعلومات عامةتاريخ البداية 1904 تعديل - تعديل مصدري - تعديل ويكي بيانات بطولات العالم للمصارعة هي بطولة عالم في رياضة مصارعة الهواة ينظمها الاتحاد العالمي لمصارعة الهواة. بدأت بطولة المصارعة اليونانية الرومانية في عام 1904.[1] والمصارعة الحرة للرجال في...

 

1986年アジア競技大会におけるバスケットボール競技(1986ねんアジアきょうぎたいかいにおけるバスケットボールきょうぎ)は9月21日から10月4日まで行われた。 男子 最終結果 順位 国 金 中国 銀 韓国 銅 フィリピン 4 ヨルダン 5 マレーシア 6 日本 7 クウェート 8 香港 女子 最終結果 順位 国 金 中国 銀 韓国 銅 日本 4 マレーシア 関連項目 アジア競技大会バスケットボー...

Historic Brahmic script This article needs additional citations for verification. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed.Find sources: Kalinga script – news · newspapers · books · scholar · JSTOR (June 2015) (Learn how and when to remove this template message) Kalinga scriptDevendravarma (Ganga), Sanskrit in Mixed Kalinga script, 9th century AD. Copper plates, exhibited...

 

This article is about string theory. For heterosis in biology, see Heterosis. String theory Fundamental objects String Cosmic string Brane D-brane Perturbative theory Bosonic Superstring (Type I, Type II, Heterotic) Non-perturbative results S-duality T-duality U-duality M-theory F-theory AdS/CFT correspondence Phenomenology Phenomenology Cosmology Landscape Mathematics Geometric Langlands correspondence Mirror symmetry Monstrous moonshine Vertex algebra Related concepts Theory of everything C...

 

American musician (born 1967) Jeff TweedyTweedy performing in 2012Background informationBirth nameJeffrey Scot TweedyBorn (1967-08-25) August 25, 1967 (age 56)Belleville, Illinois, U.S.GenresAlternative countryalternative rockOccupation(s)Musiciansongwriterauthorrecord producerInstrument(s)VocalsguitarbassharmonicaprogrammingYears active1984–presentLabelsGiant/RockvilleSireRepriseNonesuchDrag CitydBpmMember ofWilcoGolden SmogTweedyFormerly ofUncle TupeloThe Minus 5Loose FurMusical arti...

Japanese feudal domain located in Harima Province Ono Domain小野藩under Tokugawa shogunate Japan1636–1871 Mon of the Hitotsuyanagi clan CapitalOno jin'yaArea • Coordinates34°50′35.9″N 134°55′57.7″E / 34.843306°N 134.932694°E / 34.843306; 134.932694 History • TypeDaimyō Historical eraEdo period• Established 1636• Disestablished 1871 Today part ofpart of Hyōgo Prefecture class=notpageimage| Location of Ono jin'yaSh...

 

This article has multiple issues. Please help improve it or discuss these issues on the talk page. (Learn how and when to remove these template messages) This article includes a list of references, related reading, or external links, but its sources remain unclear because it lacks inline citations. Please help to improve this article by introducing more precise citations. (May 2015) (Learn how and when to remove this template message) The topic of this article may not meet Wikipedia's notabil...

 

Millennium Tower Amsterdam Millennium Tower is a 97.5 metres (320 ft), 24 storey office building in Amsterdam constructed from 2002 and completed in 2004. External links Millennium Tower at Skyscraper City vteTallest buildings in the NetherlandsChurch towers Sint Cunerachurch (Rhenen) (81.8 m) Grote of Sint-Jacobskerk (The Hague) (92.5) Sint Eusebiuschurch (Arnhem) (93 m) Sint Jacobuschurch (The Hague) (94 m) Martinichurch (Doesburg) (94 m) Martinitower (Groningen) (96.8 m) Great Church ...

Southeast, Washington D.C. high school The Digital Pioneers Academy is an information technology-focused high school in Southeast, Washington D.C.[1][2] The free public charter school is led by Mashea Ashton, the founder and Chief Executive Officer.[1] The school is focused on teaching students modern technology and innovation.[3] Most of the students are from the under-resourced areas east of the Anacostia River in Wards 7 and 8; spaces at the school are award...

 

United States Navy Q-ship This article has multiple issues. Please help improve it or discuss these issues on the talk page. (Learn how and when to remove these template messages) This article needs additional citations for verification. Please help improve this article by adding citations to reliable sources. Unsourced material may be challenged and removed.Find sources: USS Atik – news · newspapers · books · scholar · JSTOR (May 2023) (Learn how and ...

 

Town in Massachusetts, United StatesSwansea, MassachusettsTownSwansea Town Hall FlagSealLocation in Bristol County in MassachusettsCoordinates: 41°44′53″N 71°11′25″W / 41.74806°N 71.19028°W / 41.74806; -71.19028CountryUnited StatesStateMassachusettsCountyBristolSettled1662IncorporatedMarch 5, 1667Government • TypeOpen town meetingArea • Total25.5 sq mi (66.1 km2) • Land23.1 sq mi (59.7 km2)&#...

Medical conditionSpastic ataxia-corneal dystrophy syndromeOther namesBedouin spastic ataxia syndrome, Mousa-Al Din-Al Nassar syndrome and Spastic ataxia-ocular anomalies syndromeSpastic ataxia-corneal dystrophy syndrome is inherited in an autosomal recessive manner Spastic ataxia-corneal dystrophy syndrome (also known as Bedouin spastic ataxia syndrome) is an autosomally resessive disease. It has been found in an inbred Bedouin family.[1] It was first described in 1986. A member of th...

 

Bone char Pills of bone char Names Other names bone charcoalbone blackivory blackanimal charcoalabaiserPigment black 9CI 77267 Identifiers CAS Number 8021-99-6 ChemSpider none ECHA InfoCard 100.029.470 EC Number 232-421-2 CompTox Dashboard (EPA) DTXSID5027693 Properties Appearance black powder Density 0.7 - 0.8 g/cm3 Solubility in water insoluble Acidity (pKa) 8.5 - 10.0 Except where otherwise noted, data are given for materials in their standard state (at 25 °C [77 °F], 100 ...

 

Handball Liga Austria Aktuelle Saison Saison 2023/24 Sportart Handball Abkürzung HLA Verband Österreichischer Handballbund Mannschaften 12 Land/Länder Osterreich Österreich Titelträger SG Handball Westwien (6) Rekordmeister Bregenz Handball (9) TV-Partner Laola1.tv Website hla.at Qualifikation zu EHF European League EHF European Cup ↓ HLA Challenge Die höchste Handballliga Österreichs heißt Handball Liga Austria. Bei den Damen wird sie mit dem Namen Women Handball Austria...

This is a Chinese name; the family name is Di. Dilraba Dilmuratدىلرەبا دىلمۇرات迪丽热巴·迪力木拉提Dilraba in 2020Born (1992-06-03) June 3, 1992 (age 32)Ürümqi, Xinjiang, ChinaNationalityChineseOther namesDilireba DilimulatiEducationBachelor of ArtsAlma materShanghai Theatre AcademyOccupationsActressModelYears active2013–presentAgentJay Walk StudioChinese nameTraditional Chinese迪麗熱巴·迪力木拉提Simplified Chinese迪丽热巴·...

 

伊雜宮伊雑宮/いざわのみや Izawa no miya社殿基本信息位置三重縣志摩市磯部町上之鄉宗教神道主祭神天照大御神御魂社格式內社(大)・皇大神宮別宮建筑详情本殿構造神明造建立时间804年以前地圖 伊雜宮(日语:伊雑宮/いざわのみや)是位在日本三重縣志摩市的神社。多稱作「いぞうぐう」,或稱作「磯部の宮」、「磯部の大神宮さん」。為式內社、志摩國一宮。...

 

Strategi Solo vs Squad di Free Fire: Cara Menang Mudah!