O-Glutarylcarnitine (BioDeep_00000170096)
human metabolite
代谢物信息卡片
化学式: C12H21NO6 (275.13688060000004)
中文名称:
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: C[N+](C)(C)CC(CC(=O)[O-])OC(=O)CCCC(=O)O
InChI: InChI=1S/C12H21NO6/c1-13(2,3)8-9(7-11(16)17)19-12(18)6-4-5-10(14)15/h9H,4-8H2,1-3H3,(H-,14,15,16,17)
描述信息
O-Glutarylcarnitine is an acylcarnitine. More specifically, it is an glutaric acid ester of carnitine. Acylcarnitines were first discovered more than 70 year ago (PMID: 13825279). It is believed that there are more than 1000 types of acylcarnitines in the human body. The general role of acylcarnitines is to transport acyl-groups (organic acids and fatty acids) from the cytoplasm into the mitochondria so that they can be broken down to produce energy. This process is known as beta-oxidation. According to a recent review [Dambrova et al. 2021, Physiological Reviews], acylcarnitines (ACs) can be classified into 9 different categories depending on the type and size of their acyl-group: 1) short-chain ACs; 2) medium-chain ACs; 3) long-chain ACs; 4) very long-chain ACs; 5) hydroxy ACs; 6) branched chain ACs; 7) unsaturated ACs; 8) dicarboxylic ACs and 9) miscellaneous ACs. Short-chain ACs have acyl-groups with two to five carbons (C2-C5), medium-chain ACs have acyl-groups with six to thirteen carbons (C6-C13), long-chain ACs have acyl-groups with fourteen to twenty once carbons (C14-C21) and very long-chain ACs have acyl groups with more than 22 carbons. O-Glutarylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine O-Glutarylcarnitine is a member of the most abundant group of carnitines in the body, comprising more than 50\\% of all acylcarnitines quantified in tissues and biofluids (PMID: 31920980). Some short-chain carnitines have been studied as supplements or treatments for a number of diseases, including neurological disorders and inborn errors of metabolism. Carnitine acetyltransferase (CrAT, EC:2.3.1.7) is responsible for the synthesis of all short-chain and short branched-chain acylcarnitines (PMID: 23485643). The study of acylcarnitines is an active area of research and it is likely that many novel acylcarnitines will be discovered in the coming years. It is also likely that many novel roles in health and disease will be uncovered. An excellent review of the current state of knowledge for acylcarnitines is available at [Dambrova et al. 2021, Physiological Reviews].
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相关代谢途径
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代谢反应
1 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(1)
- Cerebral organic acidurias, including diseases:
L-2-Aminoadipic acid ⟶ 2-Oxoadipic acid
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
1 个相关的物种来源信息
在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有:
- PubMed: 来源于PubMed文献库中的文献信息,我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表,这个列表按照代谢物同时出现的文献数量降序排序,取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
- NCBI Taxonomy: 通过文献数据挖掘,得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序,取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
- Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
- Chemical Reaction: 在化学反应过程中,存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。
点击图上的相关代谢物的名称,可以跳转到相关代谢物的信息页面。
文献列表
- Tanyel Zubarioglu, Saffa Ahmadzada, Cengiz Yalcinkaya, Ertugrul Kiykim, Cigdem Aktuglu-Zeybek. COVID-19 triggered encephalopathic crisis in a patient with glutaric aciduria type 1.
Journal of pediatric endocrinology & metabolism : JPEM.
2021 Dec; 34(12):1611-1614. doi:
10.1515/jpem-2021-0474
. [PMID: 34517439] - Juliette Bouchereau, Manuel Schiff. Inherited Disorders of Lysine Metabolism: A Review.
The Journal of nutrition.
2020 10; 150(Suppl 1):2556S-2560S. doi:
10.1093/jn/nxaa112
. [PMID: 33000154] - Moshe Herskovitz, Dorith Goldsher, Ben-Ami Sela, Hanna Mandel. Subependymal mass lesions and peripheral polyneuropathy in adult-onset glutaric aciduria type I.
Neurology.
2013 Aug; 81(9):849-50. doi:
10.1212/wnl.0b013e3182a2cbf2
. [PMID: 23884036] - Ma Luz Couce, Olalla López-Suárez, Ma Dolores Bóveda, Daisy E Castiñeiras, José A Cocho, Judith García-Villoria, Manuel Castro-Gago, José Ma Fraga, Antonia Ribes. Glutaric aciduria type I: outcome of patients with early- versus late-diagnosis.
European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society.
2013 Jul; 17(4):383-9. doi:
10.1016/j.ejpn.2013.01.003
. [PMID: 23395213] - Tereza Moore, Anthony Le, Tina M Cowan. An improved LC-MS/MS method for the detection of classic and low excretor glutaric acidemia type 1.
Journal of inherited metabolic disease.
2012 May; 35(3):431-5. doi:
10.1007/s10545-011-9405-6
. [PMID: 22005781] - Sven W Sauer, Silvana Opp, Georg F Hoffmann, David M Koeller, Jürgen G Okun, Stefan Kölker. Therapeutic modulation of cerebral L-lysine metabolism in a mouse model for glutaric aciduria type I.
Brain : a journal of neurology.
2011 Jan; 134(Pt 1):157-70. doi:
10.1093/brain/awq269
. [PMID: 20923787] - Julia B Hennermann, Sylvia Roloff, Jutta Gellermann, Annette Grüters, Jeannette Klein. False-positive newborn screening mimicking glutaric aciduria type I in infants with renal insufficiency.
Journal of inherited metabolic disease.
2009 Dec; 32 Suppl 1(?):S355-9. doi:
10.1007/s10545-009-9017-6
. [PMID: 20033292] - Eric A Crombez, Stephen D Cederbaum, Elaine Spector, Erica Chan, Denise Salazar, Julie Neidich, Stephen Goodman. Maternal glutaric acidemia, type I identified by newborn screening.
Molecular genetics and metabolism.
2008 May; 94(1):132-4. doi:
10.1016/j.ymgme.2008.01.005
. [PMID: 18304851] - M Lindner, S Ho, J Fang-Hoffmann, G F Hoffmann, S Kölker. Neonatal screening for glutaric aciduria type I: strategies to proceed.
Journal of inherited metabolic disease.
2006 Apr; 29(2-3):378-82. doi:
10.1007/s10545-006-0284-1
. [PMID: 16763905] - Renata C Gallagher, Tina M Cowan, Stephen I Goodman, Gregory M Enns. Glutaryl-CoA dehydrogenase deficiency and newborn screening: retrospective analysis of a low excretor provides further evidence that some cases may be missed.
Molecular genetics and metabolism.
2005 Nov; 86(3):417-20. doi:
10.1016/j.ymgme.2005.08.005
. [PMID: 16183314] - S Tortorelli, S H Hahn, T M Cowan, T G Brewster, P Rinaldo, D Matern. The urinary excretion of glutarylcarnitine is an informative tool in the biochemical diagnosis of glutaric acidemia type I.
Molecular genetics and metabolism.
2005 Feb; 84(2):137-43. doi:
10.1016/j.ymgme.2004.09.016
. [PMID: 15670719] - Wei-De Lin, Chung-Hsing Wang, Chien-Chen Lai, Cheng-Chung Lee, Fuu-Jen Tsai. Genetic and biochemical study in a patient with glutaric acidemia type I.
Journal of the Formosan Medical Association = Taiwan yi zhi.
2004 Jul; 103(7):549-54. doi:
NULL
. [PMID: 15318278] - S Kölker, G F Hoffmann, D S M Schor, P Feyh, L Wagner, I Jeffrey, M Pourfarzam, J G Okun, J Zschocke, I Baric, M D Bain, C Jakobs, R A Chalmers. Glutaryl-CoA dehydrogenase deficiency: region-specific analysis of organic acids and acylcarnitines in post mortem brain predicts vulnerability of the putamen.
Neuropediatrics.
2003 Jun; 34(5):253-60. doi:
10.1055/s-2003-43261
. [PMID: 14598231] - A Ribes, E Riudor, P Briones, E Christensen, J Campistol, D S Millington. Significance of bound glutarate in the diagnosis of glutaric aciduria type I.
Journal of inherited metabolic disease.
1992; 15(3):367-70. doi:
10.1007/bf02435978
. [PMID: 1405471] - T Sakuma, N Sugiyama, T Ichiki, M Kobayashi, Y Wada, D Nohara. Analysis of acylcarnitines in maternal urine for prenatal diagnosis of glutaric aciduria type 2.
Prenatal diagnosis.
1991 Feb; 11(2):77-82. doi:
10.1002/pd.1970110203
. [PMID: 2062823] - K Kidouchi, N Sugiyama, H Morishita, Y Wada, S Nagai, J Sakakibara. Analytical method for urinary glutarylcarnitine, acetylcarnitine and propionylcarnitine with a carboxylic acid analyser and a reversed-phase column.
Journal of chromatography.
1987 Dec; 423(?):297-303. doi:
10.1016/0378-4347(87)80354-7
. [PMID: 3443662] - K Kidouchi, N Sugiyama, H Morishita, M Kobayashi, Y Wada, D Nohara. Identification of glutarylcarnitine in glutaric aciduria type 1 by carboxylic acid analyzer with an ODS reverse-phase column.
Clinica chimica acta; international journal of clinical chemistry.
1987 May; 164(3):261-6. doi:
10.1016/0009-8981(87)90300-7
. [PMID: 3594917]