succinate-CoA ligase GDP/ADP-forming subunit alpha
Ultimo aggiornamento 19 Lug 2024
Rispetta le linee guida editoriali
- ScaricaScarica
- Condividi
- Language
- Discussione
- Versione audio
- Aggiungi alle fonti preferite su Google
The SUCLG1 gene provides instructions for making one part (the alpha subunit) of an enzyme called succinyl-CoA ligase. The alpha subunit is used to make two slightly different versions of this enzyme. One version is most active in tissues such as the brain and muscle that require a large amount of energy, while the other version is most active in tissues that require less energy.
Normal function
The SUCLG1 gene provides instructions for making one part (the alpha subunit) of an enzyme called succinyl-CoA ligase. The alpha subunit is used to make two slightly different versions of this enzyme. One version is most active in tissues such as the brain and muscle that require a large amount of energy, while the other version is most active in tissues that require less energy.
Both versions of succinyl-CoA ligase play an important role in mitochondria, which are the energy-producing centers inside the cell. These enzymes are involved in a series of chemical reactions known as the citric acid cycle (or Krebs cycle). These reactions allow cells to use oxygen and produce energy.
Mitochondria each contain a small amount of DNA, known as mitochondrial DNA or mtDNA. Studies suggest that succinyl-CoA ligase interacts with another enzyme, called nucleoside diphosphate kinase, to produce and maintain the molecules that make up mtDNA. Having an adequate amount of mtDNA is essential for normal energy production within cells.
Conditions referring to this gene or chromosome
Altri nomi
GALPHA
succinate-CoA ligase, ADP-forming, alpha subunit
succinate-CoA ligase, GDP-forming, alpha subunit
succinyl-CoA synthetase, alpha subunit
SUCLA1
Related patient information and medicines
Patient information leaflet
Anticoagulanti
Anticoagulants are medicines that prevent the blood from clotting as quickly or as effectively as normal. Some people call anticoagulants blood thinners. However, the blood is not actually made any thinner - it just does not clot so easily whilst you take an anticoagulant. Anticoagulants are used to treat and prevent blood clots forming in blood.
Patient information leaflet
Artrite reumatoide
Trattamenti includono disease-modifying medicines per suppress inflammation, which can prevent or delay the progression of the disease, and medication to ease pain. Surgery is needed in some cases if a joint becomes badly damaged. See the separate leaflet called Juvenile Idiopathic Arthritis. Surrounding ligaments and muscles also help to give support.
Patient information leaflet
Test di coagulazione del sangue
Test di coagulazione del sangue are used to diagnose and assess bleeding problems and to monitor people who take warfarin o altro anticoagulant medicines. You may be advised to have blood clotting tests: If you have a suspected bleeding disorder. For example, if you bleed a lot after cuts, or if you bruise easily. If you have certain liver diseases that can.
Patient information leaflet
Uveite
The uveal tract is the name given to the part of your eye that is made up of: The iris : the part of your eye that gives it colour. The ciliary body : a small ring-like muscle that sits behind your iris and which helps the eye to focus. People who have chronic or recurrent uveitis are us... With prompt treatment, inflammation can be controlled and.
Patient information leaflet
Artrite psoriasica
In some cases, affected joints become damaged which can cause disability. Treatments include medication to ease pain, and medication to slow down the progression of the disease. Surgery is sometimes needed if a joint or tendon becomes badly damaged. What is psoriatic arthritis. Swelling and redness around the affected joints. The stiffness is.
Patient information leaflet
Spondilite anchilosante
Spondilite anchilosante è a chronic inflammatory arthritis. What is ankylosing spondylitis. Ankylosing spondylitis (AS) is a persistent (chronic) inflammatory arthritic disease. The word ankylosing means joining together or fusing. Ankylosing spondylitis is an auto-immune disease. It is not known exactly why ankylosing spondylitis occurs but there.
Ulteriori letture e riferimenti
- succinate-CoA ligase GDP/ADP-forming subunit alpha; MedlinePlus, National Library of Medicine.
- Carrozzo R, Verrigni D, Rasmussen M, de Coo R, Amartino H, Bianchi M, Buhas D, Mesli S, Naess K, Born AP, Woldseth B, Prontera P, Batbayli M, Ravn K, Joensen F, Cordelli DM, Santorelli FM, Tulinius M, Darin N, Duno M, Jouvencel P, Burlina A, Stangoni G, Bertini E, Redonnet-Vernhet I, Wibrand F, Dionisi-Vici C, Uusimaa J, Vieira P, Osorio AN, McFarland R, Taylor RW, Holme E, Ostergaard E. Succinate-CoA ligase deficiency due to mutations in SUCLA2 and SUCLG1: phenotype and genotype correlations in 71 patients. J Inherit Metab Dis. 2016 Mar;39(2):243-52. doi: 10.1007/s10545-015-9894-9. Epub 2015 Oct 16.
- El-Hattab AW, Scaglia F. SUCLG1-Related Mitochondrial DNA Depletion Syndrome, Encephalomyopathic Form with Methylmalonic Aciduria. 2017 Mar 30. In: Adam MP, Bick S, Mirzaa GM, Wallace SE, Amemiya A, editors. GeneReviews(R) [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2026. Available from http://www.ncbi.nlm.nih.gov/books/NBK425223/
- Kowluru A, Tannous M, Chen HQ. Localization and characterization of the mitochondrial isoform of the nucleoside diphosphate kinase in the pancreatic beta cell: evidence for its complexation with mitochondrial succinyl-CoA synthetase. Arch Biochem Biophys. 2002 Feb 15;398(2):160-9. doi: 10.1006/abbi.2001.2710.
- Lambeth DO, Tews KN, Adkins S, Frohlich D, Milavetz BI. Expression of two succinyl-CoA synthetases with different nucleotide specificities in mammalian tissues. J Biol Chem. 2004 Aug 27;279(35):36621-4. doi: 10.1074/jbc.M406884200. Epub 2004 Jul 2.
- Ostergaard E, Christensen E, Kristensen E, Mogensen B, Duno M, Shoubridge EA, Wibrand F. Deficiency of the alpha subunit of succinate-coenzyme A ligase causes fatal infantile lactic acidosis with mitochondrial DNA depletion. Am J Hum Genet. 2007 Aug;81(2):383-7. doi: 10.1086/519222. Epub 2007 Jun 4.
- Ostergaard E, Schwartz M, Batbayli M, Christensen E, Hjalmarson O, Kollberg G, Holme E. A novel missense mutation in SUCLG1 associated with mitochondrial DNA depletion, encephalomyopathic form, with methylmalonic aciduria. Eur J Pediatr. 2010 Feb;169(2):201-5. doi: 10.1007/s00431-009-1007-z. Epub 2009 Jun 14.
- Ostergaard E. Disorders caused by deficiency of succinate-CoA ligase. J Inherit Metab Dis. 2008 Apr;31(2):226-9. doi: 10.1007/s10545-008-0828-7. Epub 2008 Apr 4.
Partecipa alla conversazione
Condividi i tuoi pensieri o esperienze con gli altri nel nostro comunità di pazienti.
Chiedi, condividi, connettiti.
Esplora le discussioni, fai domande e condividi esperienze su centinaia di argomenti di salute.
Iscriviti alla newsletter di Patient
La tua dose settimanale di consigli sulla salute chiari e affidabili - scritti per aiutarti a sentirti informato, sicuro e in controllo.
Abbonandoti accetti i nostri Informativa sulla Privacy. Puoi annullare l'iscrizione in qualsiasi momento. Non vendiamo mai i tuoi dati.