Butyryl-CoA (BioDeep_00001868839)
Main id: BioDeep_00000001326
PANOMIX_OTCML-2023
代谢物信息卡片
Butyryl-CoA
化学式: C25H42N7O17P3S (837.1570672000001)
中文名称:
谱图信息:
最多检出来源 () 0%
分子结构信息
SMILES: CCCC(=O)SCCNC(=O)CCNC(=O)C(C(C)(C)COP(=O)(O)OP(=O)(O)OCC1C(C(C(O1)N2C=NC3=C(N=CN=C32)N)O)OP(=O)(O)O)O
InChI: InChI=1S/C25H42N7O17P3S/c1-4-5-16(34)53-9-8-27-15(33)6-7-28-23(37)20(36)25(2,3)11-46-52(43,44)49-51(41,42)45-10-14-19(48-50(38,39)40)18(35)24(47-14)32-13-31-17-21(26)29-12-30-22(17)32/h12-14,18-20,24,35-36H,4-11H2,1-3H3,(H,27,33)(H,28,37)(H,41,42)(H,43,44)(H2,26,29,30)(H2,38,39,40)/t14-,18-,19-,20+,24-/m1/s1
描述信息
A short-chain fatty acyl-CoA that results from the formal condensation of the thiol group of coenzyme A with the carboxy group of butyric acid.
同义名列表
1 个代谢物同义名
数据库引用编号
11 个数据库交叉引用编号
- ChEBI: CHEBI:15517
- KEGG: C00136
- PubChem: 122283
- CAS: 2140-48-9
- MetaboLights: MTBLC15517
- PubChem: 3436
- LipidMAPS: LMFA07050292
- PDB-CCD: BCO
- 3DMET: B04638
- NIKKAJI: J363.721B
- RefMet: Butyryl-CoA
分类词条
相关代谢途径
Reactome(0)
BioCyc(0)
PlantCyc(0)
代谢反应
0 个相关的代谢反应过程信息。
Reactome(0)
BioCyc(0)
WikiPathways(0)
Plant Reactome(0)
INOH(0)
PlantCyc(0)
COVID-19 Disease Map(0)
PathBank(0)
PharmGKB(0)
2 个相关的物种来源信息
- 9606 Homo sapiens: 10.1007/S11306-016-1051-4
- 9606 Homo sapiens: 10.1007/S11306-016-1051-4
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
- Aasia Khaliq, Resmi Ravindran, Samia Afzal, Prasant Kumar Jena, Muhammad Waheed Akhtar, Atiqa Ambreen, Yu-Jui Yvonne Wan, Kauser Abdulla Malik, Muhammad Irfan, Imran H Khan. Gut microbiome dysbiosis and correlation with blood biomarkers in active-tuberculosis in endemic setting.
PloS one.
2021; 16(1):e0245534. doi:
10.1371/journal.pone.0245534. [PMID: 33481833] - Christopher B Eiben, Tian Tian, Mitchell G Thompson, Daniel Mendez-Perez, Nurgul Kaplan, Garima Goyal, Jennifer Chiniquy, Nathan J Hillson, Taek Soon Lee, Jay D Keasling. Adenosine Triphosphate and Carbon Efficient Route to Second Generation Biofuel Isopentanol.
ACS synthetic biology.
2020 03; 9(3):468-474. doi:
10.1021/acssynbio.9b00402. [PMID: 32149502] - Zhiqiang Wen, Rodrigo Ledesma-Amaro, Minrui Lu, Mingjie Jin, Sheng Yang. Metabolic Engineering of Clostridium cellulovorans to Improve Butanol Production by Consolidated Bioprocessing.
ACS synthetic biology.
2020 02; 9(2):304-315. doi:
10.1021/acssynbio.9b00331. [PMID: 31940438] - Thi Thuy Tien Nguyen, Yuta Fujimura, Iyo Mimura, Yusuke Fujii, Ngoc Luong Nguyen, Kensuke Arakawa, Hidetoshi Morita. Cultivable butyrate-producing bacteria of elderly Japanese diagnosed with Alzheimer's disease.
Journal of microbiology (Seoul, Korea).
2018 Oct; 56(10):760-771. doi:
10.1007/s12275-018-8297-7. [PMID: 30136260] - Julian Trachsel, Darrell O Bayles, Torey Looft, Uri Y Levine, Heather K Allen. Function and Phylogeny of Bacterial Butyryl Coenzyme A:Acetate Transferases and Their Diversity in the Proximal Colon of Swine.
Applied and environmental microbiology.
2016 11; 82(22):6788-6798. doi:
10.1128/aem.02307-16. [PMID: 27613689] - Shunsuke Sato, Hiroyuki Maruyama, Tetsuya Fujiki, Keiji Matsumoto. Regulation of 3-hydroxyhexanoate composition in PHBH synthesized by recombinant Cupriavidus necator H16 from plant oil by using butyrate as a co-substrate.
Journal of bioscience and bioengineering.
2015 Sep; 120(3):246-51. doi:
10.1016/j.jbiosc.2015.01.016. [PMID: 25805434] - Randy W Purves, Stephen J Ambrose, Shawn M Clark, Jake M Stout, Jonathan E Page. Separation of isomeric short-chain acyl-CoAs in plant matrices using ultra-performance liquid chromatography coupled with tandem mass spectrometry.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences.
2015 Feb; 980(?):1-7. doi:
10.1016/j.jchromb.2014.12.007. [PMID: 25553535] - Pieter Van den Abbeele, Clara Belzer, Margot Goossens, Michiel Kleerebezem, Willem M De Vos, Olivier Thas, Rosemarie De Weirdt, Frederiek-Maarten Kerckhof, Tom Van de Wiele. Butyrate-producing Clostridium cluster XIVa species specifically colonize mucins in an in vitro gut model.
The ISME journal.
2013 May; 7(5):949-61. doi:
10.1038/ismej.2012.158. [PMID: 23235287] - Natalie M Gallant, Karen Leydiker, Hao Tang, Lisa Feuchtbaum, Fred Lorey, Rebecca Puckett, Joshua L Deignan, Julie Neidich, Naghmeh Dorrani, Erica Chang, Bruce A Barshop, Stephen D Cederbaum, Jose E Abdenur, Raymond Y Wang. Biochemical, molecular, and clinical characteristics of children with short chain acyl-CoA dehydrogenase deficiency detected by newborn screening in California.
Molecular genetics and metabolism.
2012 May; 106(1):55-61. doi:
10.1016/j.ymgme.2012.02.007. [PMID: 22424739] - Tingting Wang, Guoxiang Cai, Yunping Qiu, Na Fei, Menghui Zhang, Xiaoyan Pang, Wei Jia, Sanjun Cai, Liping Zhao. Structural segregation of gut microbiota between colorectal cancer patients and healthy volunteers.
The ISME journal.
2012 Feb; 6(2):320-9. doi:
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PloS one.
2012; 7(1):e29913. doi:
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International journal of molecular sciences.
2012; 13(8):9673-9691. doi:
10.3390/ijms13089673. [PMID: 22949824] - Carole L Linster, Gaëtane Noël, Vincent Stroobant, Didier Vertommen, Marie-Françoise Vincent, Guido T Bommer, Maria Veiga-da-Cunha, Emile Van Schaftingen. Ethylmalonyl-CoA decarboxylase, a new enzyme involved in metabolite proofreading.
The Journal of biological chemistry.
2011 Dec; 286(50):42992-3003. doi:
10.1074/jbc.m111.281527. [PMID: 22016388] - Nadja Schulz, Heinz Himmelbauer, Michaela Rath, Michel van Weeghel, Sander Houten, Wim Kulik, Karsten Suhre, Stephan Scherneck, Heike Vogel, Reinhart Kluge, Petra Wiedmer, Hans-Georg Joost, Annette Schürmann. Role of medium- and short-chain L-3-hydroxyacyl-CoA dehydrogenase in the regulation of body weight and thermogenesis.
Endocrinology.
2011 Dec; 152(12):4641-51. doi:
10.1210/en.2011-1547. [PMID: 21990309] - Berit Hippe, Jutta Zwielehner, Kathrin Liszt, Cornelia Lassl, Frank Unger, Alexander G Haslberger. Quantification of butyryl CoA:acetate CoA-transferase genes reveals different butyrate production capacity in individuals according to diet and age.
FEMS microbiology letters.
2011 Mar; 316(2):130-5. doi:
10.1111/j.1574-6968.2010.02197.x. [PMID: 21204931] - Shiyuan Hu, Huajun Zheng, Yang Gu, Jingbo Zhao, Weiwen Zhang, Yunliu Yang, Shengyue Wang, Guoping Zhao, Sheng Yang, Weihong Jiang. Comparative genomic and transcriptomic analysis revealed genetic characteristics related to solvent formation and xylose utilization in Clostridium acetobutylicum EA 2018.
BMC genomics.
2011 Feb; 12(?):93. doi:
10.1186/1471-2164-12-93. [PMID: 21284892] - B T van Maldegem, S F Kloosterman, W J Janssen, P B Augustijn, J H van der Lee, L Ijlst, H R Waterham, R Duran, R J A Wanders, F A Wijburg. High prevalence of short-chain acyl-CoA dehydrogenase deficiency in the Netherlands, but no association with epilepsy of unknown origin in childhood.
Neuropediatrics.
2011 Feb; 42(1):13-7. doi:
10.1055/s-0031-1275342. [PMID: 21500142] - Malene Hornbak, Karina Banasik, Johanne M Justesen, Nikolaj T Krarup, Camilla H Sandholt, Åsa Andersson, Annelli Sandbæk, Torsten Lauritzen, Charlotta Pisinger, Daniel R Witte, Thorkild A A Sørensen, Oluf Pedersen, Torben Hansen. The minor C-allele of rs2014355 in ACADS is associated with reduced insulin release following an oral glucose load.
BMC medical genetics.
2011 Jan; 12(?):4. doi:
10.1186/1471-2350-12-4. [PMID: 21211036] - Alain Perret, Christophe Lechaplais, Sabine Tricot, Nadia Perchat, Carine Vergne, Christine Pellé, Karine Bastard, Annett Kreimeyer, David Vallenet, Anne Zaparucha, Jean Weissenbach, Marcel Salanoubat. A novel acyl-CoA beta-transaminase characterized from a metagenome.
PloS one.
2011; 6(8):e22918. doi:
10.1371/journal.pone.0022918. [PMID: 21826218] - Oliver Fiehn, W Timothy Garvey, John W Newman, Kerry H Lok, Charles L Hoppel, Sean H Adams. Plasma metabolomic profiles reflective of glucose homeostasis in non-diabetic and type 2 diabetic obese African-American women.
PloS one.
2010 Dec; 5(12):e15234. doi:
10.1371/journal.pone.0015234. [PMID: 21170321] - Ronald J A Wanders, Jos P N Ruiter, Lodewijk IJLst, Hans R Waterham, Sander M Houten. The enzymology of mitochondrial fatty acid beta-oxidation and its application to follow-up analysis of positive neonatal screening results.
Journal of inherited metabolic disease.
2010 Oct; 33(5):479-94. doi:
10.1007/s10545-010-9104-8. [PMID: 20490924] - Bianca T van Maldegem, Ronald J A Wanders, Frits A Wijburg. Clinical aspects of short-chain acyl-CoA dehydrogenase deficiency.
Journal of inherited metabolic disease.
2010 Oct; 33(5):507-11. doi:
10.1007/s10545-010-9080-z. [PMID: 20429031] - Wenjing Jia, Rebekah N Whitehead, Lesley Griffiths, Claire Dawson, Rosemary H Waring, David B Ramsden, John O Hunter, Jeffrey A Cole. Is the abundance of Faecalibacterium prausnitzii relevant to Crohn's disease?.
FEMS microbiology letters.
2010 Sep; 310(2):138-44. doi:
10.1111/j.1574-6968.2010.02057.x. [PMID: 20695899] - Alexis Samba Mialoundama, Dimitri Heintz, Nurul Jadid, Paul Nkeng, Alain Rahier, Jozsef Deli, Bilal Camara, Florence Bouvier. Characterization of plant carotenoid cyclases as members of the flavoprotein family functioning with no net redox change.
Plant physiology.
2010 Jul; 153(3):970-9. doi:
10.1104/pp.110.155440. [PMID: 20460582] - Kenichiro Shirao, Satoshi Okada, Go Tajima, Miyuki Tsumura, Keiichi Hara, Shin'ichiro Yasunaga, Motoaki Ohtsubo, Ikue Hata, Nobuo Sakura, Yosuke Shigematsu, Yoshihiro Takihara, Masao Kobayashi. Molecular pathogenesis of a novel mutation, G108D, in short-chain acyl-CoA dehydrogenase identified in subjects with short-chain acyl-CoA dehydrogenase deficiency.
Human genetics.
2010 Jun; 127(6):619-28. doi:
10.1007/s00439-010-0822-7. [PMID: 20376488] - Christina B Pedersen, Zarazuela Zolkipli, Søren Vang, Johan Palmfeldt, Margrethe Kjeldsen, Vibeke Stenbroen, Stinne P Schmidt, Ronald J A Wanders, Jos P N Ruiter, Flemming Wibrand, Ingrid Tein, Niels Gregersen. Antioxidant dysfunction: potential risk for neurotoxicity in ethylmalonic aciduria.
Journal of inherited metabolic disease.
2010 Jun; 33(3):211-22. doi:
10.1007/s10545-010-9086-6. [PMID: 20443061] - S P Schmidt, T J Corydon, C B Pedersen, P Bross, N Gregersen. Misfolding of short-chain acyl-CoA dehydrogenase leads to mitochondrial fission and oxidative stress.
Molecular genetics and metabolism.
2010 Jun; 100(2):155-62. doi:
10.1016/j.ymgme.2010.03.009. [PMID: 20371198] - Bianca T van Maldegem, Marinus Duran, Ronald J A Wanders, Hans R Waterham, Frits A Wijburg. Flavin adenine dinucleotide status and the effects of high-dose riboflavin treatment in short-chain acyl-CoA dehydrogenase deficiency.
Pediatric research.
2010 Mar; 67(3):304-8. doi:
10.1203/pdr.0b013e3181cbd57b. [PMID: 19952864] - Patrícia Fernanda Schuck, Estela Natacha Brandt Busanello, Alana Pimentel Moura, Anelise Miotti Tonin, Mateus Grings, Luciana Ritter, Carmen Regla Vargas, Gustavo da Costa Ferreira, Moacir Wajner. Promotion of lipid and protein oxidative damage in rat brain by ethylmalonic acid.
Neurochemical research.
2010 Feb; 35(2):298-305. doi:
10.1007/s11064-009-0055-0. [PMID: 19757035] - Hisami Shinohara, Jian Wu, Toshiaki Aoyama. Effect of randomly interesterified triacylglycerol containing medium- and long-chain fatty acids on hepatic fatty acid oxidation after a single administration to rats.
Bioscience, biotechnology, and biochemistry.
2010; 74(11):2336-8. doi:
10.1271/bbb.100412. [PMID: 21071855] - Bianca T van Maldegem, Marinus Duran, Ronald J A Wanders, Hans R Waterham, Tom J de Koning, Estela Rubio, Frits A Wijburg. Fasting and fat-loading tests provide pathophysiological insight into short-chain acyl-coenzyme a dehydrogenase deficiency.
The Journal of pediatrics.
2010 Jan; 156(1):121-7. doi:
10.1016/j.jpeds.2009.07.008. [PMID: 19800078] - Laura R Jarboe, Xueli Zhang, Xuan Wang, Jonathan C Moore, K T Shanmugam, Lonnie O Ingram. Metabolic engineering for production of biorenewable fuels and chemicals: contributions of synthetic biology.
Journal of biomedicine & biotechnology.
2010; 2010(?):761042. doi:
10.1155/2010/761042. [PMID: 20414363] - Syed Imteyaz Alam, Sunita Bansod, Ravi Bhushan Kumar, Nabonita Sengupta, Lokendra Singh. Differential proteomic analysis of Clostridium perfringens ATCC13124; identification of dominant, surface and structure associated proteins.
BMC microbiology.
2009 Aug; 9(?):162. doi:
10.1186/1471-2180-9-162. [PMID: 19664283] - Julie H Rennison, Tracy A McElfresh, Xiaoqin Chen, Vijay R Anand, Brian D Hoit, Charles L Hoppel, Margaret P Chandler. Prolonged exposure to high dietary lipids is not associated with lipotoxicity in heart failure.
Journal of molecular and cellular cardiology.
2009 Jun; 46(6):883-90. doi:
10.1016/j.yjmcc.2009.02.019. [PMID: 19265702] - Reena Jethva, Can Ficicioglu. Clinical outcomes of infants with short-chain acyl-coenzyme A dehydrogenase deficiency (SCADD) detected by newborn screening.
Molecular genetics and metabolism.
2008 Dec; 95(4):241-2. doi:
10.1016/j.ymgme.2008.09.003. [PMID: 18951053] - Christina B Pedersen, Steen Kølvraa, Agnete Kølvraa, Vibeke Stenbroen, Margrethe Kjeldsen, Regina Ensenauer, Ingrid Tein, Dietrich Matern, Piero Rinaldo, Christine Vianey-Saban, Antonia Ribes, Willy Lehnert, Ernst Christensen, Thomas J Corydon, Brage S Andresen, Søren Vang, Lars Bolund, Jerry Vockley, Peter Bross, Niels Gregersen. The ACADS gene variation spectrum in 114 patients with short-chain acyl-CoA dehydrogenase (SCAD) deficiency is dominated by missense variations leading to protein misfolding at the cellular level.
Human genetics.
2008 Aug; 124(1):43-56. doi:
10.1007/s00439-008-0521-9. [PMID: 18523805] - Massimo Bionaz, Juan J Loor. Gene networks driving bovine milk fat synthesis during the lactation cycle.
BMC genomics.
2008 Jul; 9(?):366. doi:
10.1186/1471-2164-9-366. [PMID: 18671863] - Cetin Okuyaz, Fatih Süheyl Ezgü, Gürsel Biberoglu, Massimo Zeviani, Valeria Tiranti, Esat Yilgör. Severe infantile hypotonia with ethylmalonic aciduria: case report.
Journal of child neurology.
2008 Jun; 23(6):703-5. doi:
10.1177/0883073807313048. [PMID: 18539996] - Stuart G Beattie, Eric Goetzman, Thomas Conlon, Sean Germain, Glenn Walter, Martha Campbell-Thompson, Dietrich Matern, Jerry Vockley, Terence R Flotte. Biochemical correction of short-chain acyl-coenzyme A dehydrogenase deficiency after portal vein injection of rAAV8-SCAD.
Human gene therapy.
2008 Jun; 19(6):579-88. doi:
10.1089/hum.2007.168. [PMID: 18500942] - C M Westermann, L Dorland, D M Votion, M G M de Sain-van der Velden, I D Wijnberg, R J A Wanders, W G M Spliet, N Testerink, R Berger, J P N Ruiter, J H van der Kolk. Acquired multiple Acyl-CoA dehydrogenase deficiency in 10 horses with atypical myopathy.
Neuromuscular disorders : NMD.
2008 May; 18(5):355-64. doi:
10.1016/j.nmd.2008.02.007. [PMID: 18406615] - Lori A Maggio-Hall, Paul Lyne, Jon A Wolff, Nancy P Keller. A single acyl-CoA dehydrogenase is required for catabolism of isoleucine, valine and short-chain fatty acids in Aspergillus nidulans.
Fungal genetics and biology : FG & B.
2008 Mar; 45(3):180-9. doi:
10.1016/j.fgb.2007.06.004. [PMID: 17656140] - Ingrid Tein, Orly Elpeleg, Bruria Ben-Zeev, Stanley H Korman, Alexander Lossos, Dorit Lev, Tally Lerman-Sagie, Esther Leshinsky-Silver, Jerry Vockley, Gerard T Berry, Anne-Marie Lamhonwah, Dietrich Matern, Charles R Roe, Niels Gregersen. Short-chain acyl-CoA dehydrogenase gene mutation (c.319C>T) presents with clinical heterogeneity and is candidate founder mutation in individuals of Ashkenazi Jewish origin.
Molecular genetics and metabolism.
2008 Feb; 93(2):179-89. doi:
10.1016/j.ymgme.2007.09.021. [PMID: 18054510] - Jörn Oliver Sass, Regina Ensenauer, Wulf Röschinger, Horst Reich, Ulrike Steuerwald, Oliver Schirrmacher, Katharina Engel, Johannes Häberle, Brage Storstein Andresen, André Mégarbané, Willy Lehnert, Johannes Zschocke. 2-Methylbutyryl-coenzyme A dehydrogenase deficiency: functional and molecular studies on a defect in isoleucine catabolism.
Molecular genetics and metabolism.
2008 Jan; 93(1):30-5. doi:
10.1016/j.ymgme.2007.09.002. [PMID: 17945527] - Dan-Victor Giurgiutiu, Lesby M Espinoza, Tim C Wood, Barbara R DuPont, Kenton R Holden. Persistent growth failure in Prader-Willi syndrome associated with short-chain acyl-CoA dehydrogenase gene variant.
Journal of child neurology.
2008 Jan; 23(1):112-7. doi:
10.1177/0883073807307979. [PMID: 18184946] - Lan Gao, William Chiou, Hua Tang, Xueheng Cheng, Heidi S Camp, David J Burns. Simultaneous quantification of malonyl-CoA and several other short-chain acyl-CoAs in animal tissues by ion-pairing reversed-phase HPLC/MS.
Journal of chromatography. B, Analytical technologies in the biomedical and life sciences.
2007 Jun; 853(1-2):303-13. doi:
10.1016/j.jchromb.2007.03.029. [PMID: 17442642] - Olivier Levillain, Gabrielle Ventura, Henri Déchaud, Maya Hobeika, Anna Meseguer, Christophe Moinard, Luc Cynober. Sex-differential expression of ornithine aminotransferase in the mouse kidney.
American journal of physiology. Renal physiology.
2007 Mar; 292(3):F1016-27. doi:
10.1152/ajprenal.00408.2006. [PMID: 17341717] - Peter Bross, Zhijie Li, Jakob Hansen, Jens Jacob Hansen, Marit Nyholm Nielsen, Thomas Juhl Corydon, Costa Georgopoulos, Debbie Ang, Jytte Banner Lundemose, Klary Niezen-Koning, Hans Eiberg, Huanming Yang, Steen Kølvraa, Lars Bolund, Niels Gregersen. Single-nucleotide variations in the genes encoding the mitochondrial Hsp60/Hsp10 chaperone system and their disease-causing potential.
Journal of human genetics.
2007; 52(1):56-65. doi:
10.1007/s10038-006-0080-7. [PMID: 17072495] - Henri Wintz, Leslie J Yoo, Alex Loguinov, Ying-Ying Wu, Jeffrey A Steevens, Ricky D Holland, Richard D Beger, Edward J Perkins, Owen Hughes, Chris D Vulpe. Gene expression profiles in fathead minnow exposed to 2,4-DNT: correlation with toxicity in mammals.
Toxicological sciences : an official journal of the Society of Toxicology.
2006 Nov; 94(1):71-82. doi:
10.1093/toxsci/kfl080. [PMID: 16917068] - B Merinero, C Pérez-Cerdá, P Ruiz Sala, I Ferrer, M J García, M Martínez Pardo, A Belanger-Quintana, J L de la Mota, E Martin-Hernández, C Vianey-Saban, C Bischoff, N Gregersen, M Ugarte. Persistent increase of plasma butyryl/isobutyrylcarnitine concentrations as marker of SCAD defect and ethylmalonic encephalopathy.
Journal of inherited metabolic disease.
2006 Oct; 29(5):685. doi:
10.1007/s10545-006-0342-8. [PMID: 16906473] - Thomas J Conlon, Glenn Walter, Renius Owen, Travis Cossette, Kirsten Erger, Greg Gutierrez, Eric Goetzman, Dietrich Matern, Jerry Vockley, Terence R Flotte. Systemic correction of a fatty acid oxidation defect by intramuscular injection of a recombinant adeno-associated virus vector.
Human gene therapy.
2006 Jan; 17(1):71-80. doi:
10.1089/hum.2006.17.71. [PMID: 16409126] - A Michele Schuler, Barbara A Gower, Dietrich Matern, Piero Rinaldo, Jerry Vockley, Philip A Wood. Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid beta-oxidation.
Molecular genetics and metabolism.
2005 May; 85(1):7-11. doi:
10.1016/j.ymgme.2004.09.006. [PMID: 15862275] - Stanley H Korman, Brage S Andresen, Avraham Zeharia, Alisa Gutman, Avihu Boneh, James J Pitt. 2-ethylhydracrylic aciduria in short/branched-chain acyl-CoA dehydrogenase deficiency: application to diagnosis and implications for the R-pathway of isoleucine oxidation.
Clinical chemistry.
2005 Mar; 51(3):610-7. doi:
10.1373/clinchem.2004.043265. [PMID: 15615815] - B T van Maldegem, H R Waterham, M Duran, M van der Vlies, C S van Woerden, L L Bobu, R J A Wanders, F A Wijburg. The 625G>A SCAD gene variant is common but not associated with increased C4-carnitine in newborn blood spots.
Journal of inherited metabolic disease.
2005; 28(4):557-62. doi:
10.1007/s10545-005-0557-0. [PMID: 15902559] - A Michele Schuler, Barbara A Gower, Dietrich Matern, Piero Rinaldo, Philip A Wood. Influence of dietary fatty acid chain-length on metabolic tolerance in mouse models of inherited defects in mitochondrial fatty acid beta-oxidation.
Molecular genetics and metabolism.
2004 Dec; 83(4):322-9. doi:
10.1016/j.ymgme.2004.08.010. [PMID: 15589119] - Tiina Tyni, Anders Paetau, Arnold W Strauss, Bruce Middleton, Tero Kivelä. Mitochondrial fatty acid beta-oxidation in the human eye and brain: implications for the retinopathy of long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency.
Pediatric research.
2004 Nov; 56(5):744-50. doi:
10.1203/01.pdr.0000141967.52759.83. [PMID: 15347768] - Christina Bak Pedersen, Peter Bross, Vibeke Stenbroen Winter, Thomas Juhl Corydon, Lars Bolund, Kim Bartlett, Jerry Vockley, Niels Gregersen. Misfolding, degradation, and aggregation of variant proteins. The molecular pathogenesis of short chain acyl-CoA dehydrogenase (SCAD) deficiency.
The Journal of biological chemistry.
2003 Nov; 278(48):47449-58. doi:
10.1074/jbc.m309514200. [PMID: 14506246] - Levinus A Bok, Peter Vreken, Frits A Wijburg, Ronald J A Wanders, Niels Gregersen, Morten J Corydon, Hans R Waterham, Marinus Duran. Short-chain Acyl-CoA dehydrogenase deficiency: studies in a large family adding to the complexity of the disorder.
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