Succinic acid semialdehyde (BioDeep_00000002862)

 

Secondary id: BioDeep_00000405430

natural product human metabolite PANOMIX_OTCML-2023 Endogenous


代谢物信息卡片


Succinic semialdehyde, calcium salt

化学式: C4H6O3 (102.0316926)
中文名称: 琥珀半醛, 琥珀半醛
谱图信息: 最多检出来源 Viridiplantae(plant) 0.05%

分子结构信息

SMILES: C(=O)CCC(=O)O
InChI: InChI=1S/C4H6O3/c5-3-1-2-4(6)7/h3H,1-2H2,(H,6,7)

描述信息

Succinic acid semialdehyde (or succinate semialdehyde) is an intermediate in the catabolism of gamma-aminobutyrate or GABA (PMID:16435183). It is formed from GABA by the action of GABA transaminase, which leads to the production of succinate semialdehyde and alanine. The resulting succinate semialdehyde is further oxidized by succinate semialdehyde dehydrogenase to become succinic acid, which also yields NADPH. Under certain situations, high levels of succinate semialdehyde can function as a neurotoxin and a metabotoxin. A neurotoxin is a compound that causes damage to the brain and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Elevated serum levels of succinate semialdehyde are found in succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria), a rare neurometabolic disorder of gamma-aminobutyric acid (GABA) degradation. Symptoms include motor delay, hypotonia, speech delay, autistic features, seizures, and ataxia. Patients also exhibit behavioural problems such as attention deficit, hyperactivity, anxiety, or aggression (PMID:18622364). Succinate semialdehyde is considered a reactive carbonyl and may lead to increased oxidative stress. This stress is believed to contribute to the formation of free radicals in the brain tissue of animal models induced with SSADH deficiency, which further leads to secondary cell damage and death. Additionally, oxidative stress may be responsible for the loss of striatal dopamine, which may contribute to the neuropathology of SSADH deficiency.
Succinic acid semialdehyde is an intermediate in the catabolism of gamma-aminobutyrate (PMID 16435183). Succinate semialdehyde dehydrogenase is an enzyme that catalyses the reaction of succinate semialdehyde and NAD+ to form succinate and NADH. Succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria) is a rare neurometabolic disorder of gamma-aminobutyric acid degradation. Symptoms include motor delay, hypotonia, speech delay, autistic features, seizures, and ataxia. Patients also exhibit behavioral problems, such as attention deficit, hyperactivity, anxiety, or aggression. (PMID: 18622364) [HMDB]. Succinic acid semialdehyde is found in many foods, some of which are yellow zucchini, japanese chestnut, banana, and pineappple sage.

同义名列表

29 个代谢物同义名

Succinic semialdehyde, calcium salt; 2-Formylpropionic acid ethyl ester; Succinic Semialdehyde solution; β-Formylpropionic acid; Succinic acid semialdehyde; beta-Formylpropionic acid; γ-Oxybutyric acid; β-Formylpropionate; Semialdehyde succinique; Β-formylpropionic acid; 3-Formylpropanoic acid; b-Formylpropionic acid; Succinate semialdehyde; 3-Formylpropionic acid; beta-Formylpropionate; gamma-Oxybutyric acid; Succinic semialdehyde; Succinaldehydic acid; γ-Oxybutyrate; Butryaldehydic acid; 3-Formylpropanoate; 4-Oxobutanoic acid; 3-Formylpropionate; b-Formylpropionate; Β-formylpropionate; gamma-Oxybutyrate; Succinaldehydate; Butryaldehydate; 4-Oxobutanoate



数据库引用编号

22 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(5)

PlantCyc(0)

代谢反应

355 个相关的代谢反应过程信息。

Reactome(14)

BioCyc(7)

WikiPathways(0)

Plant Reactome(300)

INOH(3)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(31)

PharmGKB(0)

5 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

  • Trevor Kirby, Dana C Walters, Madalyn Brown, Erwin Jansen, Gajja S Salomons, Coleman Turgeon, Piero Rinaldo, Erland Arning, Paula Ashcraft, Teodoro Bottiglieri, Jean-Baptiste Roullet, K Michael Gibson. Post-mortem tissue analyses in a patient with succinic semialdehyde dehydrogenase deficiency (SSADHD). I. Metabolomic outcomes. Metabolic brain disease. 2020 04; 35(4):601-614. doi: 10.1007/s11011-020-00550-1. [PMID: 32172518]
  • Stephany Angelia Tumewu, Hidenori Matsui, Mikihiro Yamamoto, Yoshiteru Noutoshi, Kazuhiro Toyoda, Yuki Ichinose. Requirement of γ-Aminobutyric Acid Chemotaxis for Virulence of Pseudomonas syringae pv. tabaci 6605. Microbes and environments. 2020; 35(4):. doi: 10.1264/jsme2.me20114. [PMID: 33162464]
  • Sara Leo, Concetta Capo, Bianca Maria Ciminelli, Federico Iacovelli, Giovanna Menduti, Silvia Funghini, Maria Alice Donati, Mattia Falconi, Luisa Rossi, Patrizia Malaspina. SSADH deficiency in an Italian family: a novel ALDH5A1 gene mutation affecting the succinic semialdehyde substrate binding site. Metabolic brain disease. 2017 10; 32(5):1383-1388. doi: 10.1007/s11011-017-0058-5. [PMID: 28664505]
  • Martina Kopečná, Armelle Vigouroux, Jan Vilím, Radka Končitíková, Pierre Briozzo, Eva Hájková, Lenka Jašková, Klaus von Schwartzenberg, Marek Šebela, Solange Moréra, David Kopečný. The ALDH21 gene found in lower plants and some vascular plants codes for a NADP+ -dependent succinic semialdehyde dehydrogenase. The Plant journal : for cell and molecular biology. 2017 Oct; 92(2):229-243. doi: 10.1111/tpj.13648. [PMID: 28749584]
  • Dereje Worku Mekonnen, Frank Ludewig. Phenotypic and chemotypic studies using Arabidopsis and yeast reveal that GHB converts to SSA and induce toxicity. Plant molecular biology. 2016 Jul; 91(4-5):429-40. doi: 10.1007/s11103-016-0475-6. [PMID: 27037708]
  • Chao Wang, Desong Tang, Yong-Gui Gao, Lian-Hui Zhang. Succinic Semialdehyde Promotes Prosurvival Capability of Agrobacterium tumefaciens. Journal of bacteriology. 2016 Jan; 198(6):930-40. doi: 10.1128/jb.00373-15. [PMID: 26755630]
  • Qi Peng, Min Yang, Wei Wang, Lili Han, Guannan Wang, Pengyue Wang, Jie Zhang, Fuping Song. Activation of gab cluster transcription in Bacillus thuringiensis by γ-aminobutyric acid or succinic semialdehyde is mediated by the Sigma 54-dependent transcriptional activator GabR. BMC microbiology. 2014 Dec; 14(?):306. doi: 10.1186/s12866-014-0306-3. [PMID: 25527261]
  • Koichi Toyokura, Makoto Hayashi, Mikio Nishimura, Kiyotaka Okada. Adaxial-abaxial patterning: a novel function of the GABA shunt. Plant signaling & behavior. 2012 Jul; 7(7):705-7. doi: 10.4161/psb.20346. [PMID: 22751326]
  • Jeffrey Sung-Shing Kwok, Chi Lap Yuen, Lap Kay Law, Nelson Leung-Sang Tang, Sharon Wan-Wah Cherk, Yuet Ping Yuen. A novel ALDH5A1 mutation in a patient with succinic semialdehyde dehydrogenase deficiency. Pathology. 2012 Apr; 44(3):280-2. doi: 10.1097/pat.0b013e32835140c2. [PMID: 22437753]
  • Ana M Fortes, Patricia Agudelo-Romero, Marta S Silva, Kashif Ali, Lisete Sousa, Federica Maltese, Young H Choi, Jerome Grimplet, José M Martinez-Zapater, Robert Verpoorte, Maria S Pais. Transcript and metabolite analysis in Trincadeira cultivar reveals novel information regarding the dynamics of grape ripening. BMC plant biology. 2011 Nov; 11(?):149. doi: 10.1186/1471-2229-11-149. [PMID: 22047180]
  • Ehud Katz, Kyung Hwan Boo, Ho Youn Kim, Richard A Eigenheer, Brett S Phinney, Vladimir Shulaev, Florence Negre-Zakharov, Avi Sadka, Eduardo Blumwald. Label-free shotgun proteomics and metabolite analysis reveal a significant metabolic shift during citrus fruit development. Journal of experimental botany. 2011 Nov; 62(15):5367-84. doi: 10.1093/jxb/err197. [PMID: 21841177]
  • Xiao-Lu Deng, Fei Yin, Qiu-Lian Xiang, Chen-Tao Liu, Jing Peng. [Succinic semialdehyde dehydrogenase deficiency]. Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics. 2011 Sep; 13(9):740-2. doi: NULL. [PMID: 21924025]
  • Kyung-Jin Kim, Phillip L Pearl, Kimmo Jensen, O Carter Snead, Patrizia Malaspina, Cornelis Jakobs, K Michael Gibson. Succinic semialdehyde dehydrogenase: biochemical-molecular-clinical disease mechanisms, redox regulation, and functional significance. Antioxidants & redox signaling. 2011 Aug; 15(3):691-718. doi: 10.1089/ars.2010.3470. [PMID: 20973619]
  • Koichi Toyokura, Keiro Watanabe, Akira Oiwaka, Miyako Kusano, Toshiaki Tameshige, Kiyoshi Tatematsu, Noritaka Matsumoto, Ryuji Tsugeki, Kazuki Saito, Kiyotaka Okada. Succinic semialdehyde dehydrogenase is involved in the robust patterning of Arabidopsis leaves along the adaxial-abaxial axis. Plant & cell physiology. 2011 Aug; 52(8):1340-53. doi: 10.1093/pcp/pcr079. [PMID: 21690177]
  • Sabrina B Cardillo, Susana Correa García, Mariana Bermúdez Moretti. Common features and differences in the expression of the three genes forming the UGA regulon in Saccharomyces cerevisiae. Biochemical and biophysical research communications. 2011 Jul; 410(4):885-9. doi: 10.1016/j.bbrc.2011.06.086. [PMID: 21708130]
  • Linda Siggberg, Aki Mustonen, Robert Schuit, Gajja S Salomons, Birthe Roos, K Michael Gibson, Cornelis Jakobs, Jaakko Ignatius, Sakari Knuutila. Familial 6p22.2 duplication associates with mild developmental delay and increased SSADH activity. American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics. 2011 Jun; 156B(4):448-53. doi: 10.1002/ajmg.b.31180. [PMID: 21438145]
  • M M C Wamelink, B Roos, E E W Jansen, M F Mulder, K M Gibson, C Jakobs. 4-Hydroxybutyric aciduria associated with catheter usage: a diagnostic pitfall in the identification of SSADH deficiency. Molecular genetics and metabolism. 2011 Feb; 102(2):216-7. doi: 10.1016/j.ymgme.2010.10.001. [PMID: 20965758]
  • Min Guo, Yue Chen, Yan Du, Yanhan Dong, Wang Guo, Su Zhai, Haifeng Zhang, Suomeng Dong, Zhengguang Zhang, Yuanchao Wang, Ping Wang, Xiaobo Zheng. The bZIP transcription factor MoAP1 mediates the oxidative stress response and is critical for pathogenicity of the rice blast fungus Magnaporthe oryzae. PLoS pathogens. 2011 Feb; 7(2):e1001302. doi: 10.1371/journal.ppat.1001302. [PMID: 21383978]
  • Fiorenzo Conti, Marcello Melone, Giorgia Fattorini, Luca Bragina, Silvia Ciappelloni. A Role for GAT-1 in Presynaptic GABA Homeostasis?. Frontiers in cellular neuroscience. 2011; 5(?):2. doi: 10.3389/fncel.2011.00002. [PMID: 21503156]
  • Naim Stiti, Tagnon D Missihoun, Simeon O Kotchoni, Hans-Hubert Kirch, Dorothea Bartels. Aldehyde Dehydrogenases in Arabidopsis thaliana: Biochemical Requirements, Metabolic Pathways, and Functional Analysis. Frontiers in plant science. 2011; 2(?):65. doi: 10.3389/fpls.2011.00065. [PMID: 22639603]
  • Carmen Espinoza, Thomas Degenkolbe, Camila Caldana, Ellen Zuther, Andrea Leisse, Lothar Willmitzer, Dirk K Hincha, Matthew A Hannah. Interaction with diurnal and circadian regulation results in dynamic metabolic and transcriptional changes during cold acclimation in Arabidopsis. PloS one. 2010 Nov; 5(11):e14101. doi: 10.1371/journal.pone.0014101. [PMID: 21124901]
  • Yong-Gen Yin, Takehiro Tominaga, Yoko Iijima, Koh Aoki, Daisuke Shibata, Hiroshi Ashihara, Shigeo Nishimura, Hiroshi Ezura, Chiaki Matsukura. Metabolic alterations in organic acids and gamma-aminobutyric acid in developing tomato (Solanum lycopersicum L.) fruits. Plant & cell physiology. 2010 Aug; 51(8):1300-14. doi: 10.1093/pcp/pcq090. [PMID: 20595461]
  • Hiromoto Yamakawa, Makoto Hakata. Atlas of rice grain filling-related metabolism under high temperature: joint analysis of metabolome and transcriptome demonstrated inhibition of starch accumulation and induction of amino acid accumulation. Plant & cell physiology. 2010 May; 51(5):795-809. doi: 10.1093/pcp/pcq034. [PMID: 20304786]
  • Martijn Kranendijk, Eduard A Struys, K Michael Gibson, Wjera V Wickenhagen, Jose E Abdenur, Jochen Buechner, Ernst Christensen, Raquel Dodelson de Kremer, Abdellatif Errami, Paul Gissen, Wanda Gradowska, Emma Hobson, Lily Islam, Stanley H Korman, Thaddeus Kurczynski, Bruno Maranda, Concetta Meli, Cristiano Rizzo, Claude Sansaricq, Friedrich K Trefz, Rachel Webster, Cornelis Jakobs, Gajja S Salomons. Evidence for genetic heterogeneity in D-2-hydroxyglutaric aciduria. Human mutation. 2010 Mar; 31(3):279-83. doi: 10.1002/humu.21186. [PMID: 20020533]
  • Marcio Rocha, Francesco Licausi, Wagner L Araújo, Adriano Nunes-Nesi, Ladaslav Sodek, Alisdair R Fernie, Joost T van Dongen. Glycolysis and the tricarboxylic acid cycle are linked by alanine aminotransferase during hypoxia induced by waterlogging of Lotus japonicus. Plant physiology. 2010 Mar; 152(3):1501-13. doi: 10.1104/pp.109.150045. [PMID: 20089769]
  • G Iglesias Escalera, I Ferrer, Ll Carrasco Marina, P Ruiz Sala, G S Salomons, C Jakobs, C Pérez-Cerdá. [Succinic semialdehyde dehydrogenase deficiency: decrease in 4-OH-butyric acid levels with low doses of vigabatrin]. Anales de pediatria (Barcelona, Spain : 2003). 2010 Feb; 72(2):128-32. doi: 10.1016/j.anpedi.2009.09.018. [PMID: 20018576]
  • Hugues Renault, Valérie Roussel, Abdelhak El Amrani, Matthieu Arzel, David Renault, Alain Bouchereau, Carole Deleu. The Arabidopsis pop2-1 mutant reveals the involvement of GABA transaminase in salt stress tolerance. BMC plant biology. 2010 Feb; 10(?):20. doi: 10.1186/1471-2229-10-20. [PMID: 20122158]
  • Wendy L Allan, Shawn M Clark, Gordon J Hoover, Barry J Shelp. Role of plant glyoxylate reductases during stress: a hypothesis. The Biochemical journal. 2009 Sep; 423(1):15-22. doi: 10.1042/bj20090826. [PMID: 19740079]
  • Patrizia Malaspina, Matthew J Picklo, C Jakobs, O Carter Snead, K Michael Gibson. Comparative genomics of aldehyde dehydrogenase 5a1 (succinate semialdehyde dehydrogenase) and accumulation of gamma-hydroxybutyrate associated with its deficiency. Human genomics. 2009 Jan; 3(2):106-20. doi: 10.1186/1479-7364-3-2-106. [PMID: 19164088]
  • Caroline B Michielse, Ringo van Wijk, Linda Reijnen, Ben J C Cornelissen, Martijn Rep. Insight into the molecular requirements for pathogenicity of Fusarium oxysporum f. sp. lycopersici through large-scale insertional mutagenesis. Genome biology. 2009; 10(1):R4. doi: 10.1186/gb-2009-10-1-r4. [PMID: 19134172]
  • Yufei Wang, Zeliang Chen, Feng Qiao, Tianyi Ying, Jing Yuan, Zhijun Zhong, Lei Zhou, Xinying Du, Zhoujia Wang, Jin Zhao, Shicun Dong, Leili Jia, Xitong Yuan, Ruifu Yang, Yansong Sun, Liuyu Huang. Comparative proteomics analyses reveal the virB of B. melitensis affects expression of intracellular survival related proteins. PloS one. 2009; 4(4):e5368. doi: 10.1371/journal.pone.0005368. [PMID: 19401764]
  • Philip A Bronstein, Melanie J Filiatrault, Christopher R Myers, Michael Rutzke, David J Schneider, Samuel W Cartinhour. Global transcriptional responses of Pseudomonas syringae DC3000 to changes in iron bioavailability in vitro. BMC microbiology. 2008 Dec; 8(?):209. doi: 10.1186/1471-2180-8-209. [PMID: 19055731]
  • Jean-Benoit F Charron, Francois Ouellet, Mario Houde, Fathey Sarhan. The plant Apolipoprotein D ortholog protects Arabidopsis against oxidative stress. BMC plant biology. 2008 Jul; 8(?):86. doi: 10.1186/1471-2229-8-86. [PMID: 18671872]
  • Ina Knerr, K Michael Gibson, Cornelis Jakobs, Phillip L Pearl. Neuropsychiatric morbidity in adolescent and adult succinic semialdehyde dehydrogenase deficiency patients. CNS spectrums. 2008 Jul; 13(7):598-605. doi: 10.1017/s1092852900016874. [PMID: 18622364]
  • Eric K Long, Tonya C Murphy, Laura J Leiphon, John Watt, Jason D Morrow, Ginger L Milne, Jocelyn R H Howard, Matthew J Picklo. Trans-4-hydroxy-2-hexenal is a neurotoxic product of docosahexaenoic (22:6; n-3) acid oxidation. Journal of neurochemistry. 2008 May; 105(3):714-24. doi: 10.1111/j.1471-4159.2007.05175.x. [PMID: 18194211]
  • Frances M Dupont. Metabolic pathways of the wheat (Triticum aestivum) endosperm amyloplast revealed by proteomics. BMC plant biology. 2008 Apr; 8(?):39. doi: 10.1186/1471-2229-8-39. [PMID: 18419817]
  • Katherine G Zulak, Aalim M Weljie, Hans J Vogel, Peter J Facchini. Quantitative 1H NMR metabolomics reveals extensive metabolic reprogramming of primary and secondary metabolism in elicitor-treated opium poppy cell cultures. BMC plant biology. 2008 Jan; 8(?):5. doi: 10.1186/1471-2229-8-5. [PMID: 18211706]
  • Wendy L Allan, Jeffrey P Simpson, Shawn M Clark, Barry J Shelp. Gamma-hydroxybutyrate accumulation in Arabidopsis and tobacco plants is a general response to abiotic stress: putative regulation by redox balance and glyoxylate reductase isoforms. Journal of experimental botany. 2008; 59(9):2555-64. doi: 10.1093/jxb/ern122. [PMID: 18495640]
  • Jeffrey P Simpson, Rosa Di Leo, Preetinder K Dhanoa, Wendy L Allan, Amina Makhmoudova, Shawn M Clark, Gordon J Hoover, Robert T Mullen, Barry J Shelp. Identification and characterization of a plastid-localized Arabidopsis glyoxylate reductase isoform: comparison with a cytosolic isoform and implications for cellular redox homeostasis and aldehyde detoxification. Journal of experimental botany. 2008; 59(9):2545-54. doi: 10.1093/jxb/ern123. [PMID: 18495639]
  • Y H Tao, D Y Jiang, H B Xu, X L Yang. Inhibitory effect of Erigeron breviscapus extract and its flavonoid components on GABA shunt enzymes. Phytomedicine : international journal of phytotherapy and phytopharmacology. 2008 Jan; 15(1-2):92-7. doi: 10.1016/j.phymed.2007.06.009. [PMID: 17689232]
  • Cecilia Vasquez-Robinet, Shrinivasrao P Mane, Alexander V Ulanov, Jonathan I Watkinson, Verlyn K Stromberg, David De Koeyer, Roland Schafleitner, David B Willmot, Merideth Bonierbale, Hans J Bohnert, Ruth Grene. Physiological and molecular adaptations to drought in Andean potato genotypes. Journal of experimental botany. 2008; 59(8):2109-23. doi: 10.1093/jxb/ern073. [PMID: 18535297]
  • A Latini, K Scussiato, G Leipnitz, K M Gibson, M Wajner. Evidence for oxidative stress in tissues derived from succinate semialdehyde dehydrogenase-deficient mice. Journal of inherited metabolic disease. 2007 Oct; 30(5):800-10. doi: 10.1007/s10545-007-0599-6. [PMID: 17885820]
  • Maarten Fauvart, Kristien Braeken, Ruth Daniels, Karen Vos, Maxime Ndayizeye, Jean-Paul Noben, Johan Robben, Jos Vanderleyden, Jan Michiels. Identification of a novel glyoxylate reductase supports phylogeny-based enzymatic substrate specificity prediction. Biochimica et biophysica acta. 2007 Sep; 1774(9):1092-8. doi: 10.1016/j.bbapap.2007.06.009. [PMID: 17693143]
  • S-J Ahn, C-H Yang, D A Cooksey. Pseudomonas putida 06909 genes expressed during colonization on mycelial surfaces and phenotypic characterization of mutants. Journal of applied microbiology. 2007 Jul; 103(1):120-32. doi: 10.1111/j.1365-2672.2006.03232.x. [PMID: 17584458]
  • I Knerr, P L Pearl, T Bottiglieri, O Carter Snead, C Jakobs, K M Gibson. Therapeutic concepts in succinate semialdehyde dehydrogenase (SSADH; ALDH5a1) deficiency (gamma-hydroxybutyric aciduria). Hypotheses evolved from 25 years of patient evaluation, studies in Aldh5a1-/- mice and characterization of gamma-hydroxybutyric acid pharmacology. Journal of inherited metabolic disease. 2007 Jun; 30(3):279-94. doi: 10.1007/s10545-007-0574-2. [PMID: 17457693]
  • G Barcelo-Coblijn, E J Murphy, K Mills, B Winchester, C Jakobs, O C Snead, K M Gibson. Lipid abnormalities in succinate semialdehyde dehydrogenase (Aldh5a1-/-) deficient mouse brain provide additional evidence for myelin alterations. Biochimica et biophysica acta. 2007 May; 1772(5):556-62. doi: 10.1016/j.bbadis.2006.12.008. [PMID: 17300923]
  • Laura J Leiphon, Matthew J Picklo. Inhibition of aldehyde detoxification in CNS mitochondria by fungicides. Neurotoxicology. 2007 Jan; 28(1):143-9. doi: 10.1016/j.neuro.2006.08.008. [PMID: 17010440]
  • Manuel Cercós, Guillermo Soler, Domingo J Iglesias, José Gadea, Javier Forment, Manuel Talón. Global analysis of gene expression during development and ripening of citrus fruit flesh. A proposed mechanism for citric Acid utilization. Plant molecular biology. 2006 Nov; 62(4-5):513-27. doi: 10.1007/s11103-006-9037-7. [PMID: 16897468]
  • Aida Lemes, Paola Blasi, Gabriel Gonzales, Maria E Russi, Roberto Quadrelli, Andrea Novelletto, Patrizia Malaspina. Succinic semialdehyde dehydrogenase (SSADH) deficiency: Molecular analysis in a South American family. Journal of inherited metabolic disease. 2006 Aug; 29(4):587. doi: 10.1007/s10545-006-0277-0. [PMID: 16788854]
  • Andrea Buzzi, Ying Wu, Marina V Frantseva, Jose L Perez Velazquez, Miguel A Cortez, Chun C Liu, Li Q Shen, K Michael Gibson, O Carter Snead. Succinic semialdehyde dehydrogenase deficiency: GABAB receptor-mediated function. Brain research. 2006 May; 1090(1):15-22. doi: 10.1016/j.brainres.2006.02.131. [PMID: 16647690]
  • E A Struys, N M Verhoeven, G S Salomons, J Berthelot, C Vianay-Saban, S Chabrier, J A Thomas, A Chun-Hui Tsai, K M Gibson, C Jakobs. D-2-hydroxyglutaric aciduria in three patients with proven SSADH deficiency: genetic coincidence or a related biochemical epiphenomenon?. Molecular genetics and metabolism. 2006 May; 88(1):53-7. doi: 10.1016/j.ymgme.2005.12.002. [PMID: 16442322]
  • Erwin E W Jansen, Nanda M Verhoeven, Cornelis Jakobs, Andreas Schulze, Henry Senephansiri, Maneesh Gupta, O Carter Snead, K Michael Gibson. Increased guanidino species in murine and human succinate semialdehyde dehydrogenase (SSADH) deficiency. Biochimica et biophysica acta. 2006 Apr; 1762(4):494-8. doi: 10.1016/j.bbadis.2006.01.006. [PMID: 16504488]
  • Eduard A Struys, Nanda M Verhoeven, Erwin E W Jansen, Herman J Ten Brink, Maneesh Gupta, Terry G Burlingame, Lawrence S Quang, Timothy Maher, Piero Rinaldo, O Carter Snead, Amy K Goodwin, Elise M Weerts, P Rand Brown, Tonya C Murphy, Mathew J Picklo, Cornelius Jakobs, K Michael Gibson. Metabolism of gamma-hydroxybutyrate to d-2-hydroxyglutarate in mammals: further evidence for d-2-hydroxyglutarate transhydrogenase. Metabolism: clinical and experimental. 2006 Mar; 55(3):353-8. doi: 10.1016/j.metabol.2005.09.009. [PMID: 16483879]
  • Jeong Han Kang, Yong Bok Park, Tae-Lin Huh, Won-Ha Lee, Myung-Sook Choi, Oh-Shin Kwon. High-level expression and characterization of the recombinant enzyme, and tissue distribution of human succinic semialdehyde dehydrogenase. Protein expression and purification. 2005 Nov; 44(1):16-22. doi: 10.1016/j.pep.2005.03.019. [PMID: 16199352]
  • Soo Young Choi, Eun-Mi Ahn, Myoung-Chong Song, Dae Won Kim, Jeong Han Kang, Oh-Shin Kwon, Tae-Cheon Kang, Nam-In Baek. In vitro GABA-transaminase inhibitory compounds from the root of Angelica dahurica. Phytotherapy research : PTR. 2005 Oct; 19(10):839-45. doi: 10.1002/ptr.1424. [PMID: 16261512]
  • Isabelle Arnulf, Eric Konofal, K Michael Gibson, Daniel Rabier, Pierre Beauvais, Jean-Philippe Derenne, Anne Philippe. Effect of genetically caused excess of brain gamma-hydroxybutyric acid and GABA on sleep. Sleep. 2005 Apr; 28(4):418-24. doi: 10.1093/sleep/28.4.418. [PMID: 16171286]
  • Aaron Fait, Ayelet Yellin, Hillel Fromm. GABA shunt deficiencies and accumulation of reactive oxygen intermediates: insight from Arabidopsis mutants. FEBS letters. 2005 Jan; 579(2):415-20. doi: 10.1016/j.febslet.2004.12.004. [PMID: 15642352]
  • E A Struys, E E W Jansen, K M Gibson, C Jakobs. Determination of the GABA analogue succinic semialdehyde in urine and cerebrospinal fluid by dinitrophenylhydrazine derivatization and liquid chromatography-tandem mass spectrometry: application to SSADH deficiency. Journal of inherited metabolic disease. 2005; 28(6):913-20. doi: 10.1007/s10545-005-0111-0. [PMID: 16435183]
  • K M Gibson. Gamma-hydroxybutyric aciduria: a biochemist's education from a heritable disorder of GABA metabolism. Journal of inherited metabolic disease. 2005; 28(3):247-65. doi: 10.1007/s10545-005-7053-4. [PMID: 15868461]
  • M A Cortez, Y Wu, K M Gibson, O C Snead. Absence seizures in succinic semialdehyde dehydrogenase deficient mice: a model of juvenile absence epilepsy. Pharmacology, biochemistry, and behavior. 2004 Nov; 79(3):547-53. doi: 10.1016/j.pbb.2004.09.008. [PMID: 15582027]
  • Aurélien Carlier, Romain Chevrot, Yves Dessaux, Denis Faure. The assimilation of gamma-butyrolactone in Agrobacterium tumefaciens C58 interferes with the accumulation of the N-acyl-homoserine lactone signal. Molecular plant-microbe interactions : MPMI. 2004 Sep; 17(9):951-7. doi: 10.1094/mpmi.2004.17.9.951. [PMID: 15384485]
  • Hans-Hubert Kirch, Dorothea Bartels, Yanling Wei, Patrick S Schnable, Andrew J Wood. The ALDH gene superfamily of Arabidopsis. Trends in plant science. 2004 Aug; 9(8):371-7. doi: 10.1016/j.tplants.2004.06.004. [PMID: 15358267]
  • A Dervent, K M Gibson, P L Pearl, G S Salomons, C Jakobs, C Yalcinkaya. Photosensitive absence epilepsy with myoclonias and heterozygosity for succinic semialdehyde dehydrogenase (SSADH) deficiency. Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology. 2004 Jun; 115(6):1417-22. doi: 10.1016/j.clinph.2004.01.002. [PMID: 15134710]
  • Maneesh Gupta, Erwin E W Jansen, Henry Senephansiri, Cornelis Jakobs, O Carter Snead, Markus Grompe, K Michael Gibson. Liver-directed adenoviral gene transfer in murine succinate semialdehyde dehydrogenase deficiency. Molecular therapy : the journal of the American Society of Gene Therapy. 2004 Apr; 9(4):527-39. doi: 10.1016/j.ymthe.2004.01.013. [PMID: 15093183]
  • Phillip L Pearl, K Michael Gibson. Clinical aspects of the disorders of GABA metabolism in children. Current opinion in neurology. 2004 Apr; 17(2):107-13. doi: 10.1097/00019052-200404000-00005. [PMID: 15021235]
  • Phillip L Pearl, Andrea Gropman. Monitoring gamma-hydroxybutyric acid levels in succinate-semialdehyde dehydrogenase deficiency. Annals of neurology. 2004 Apr; 55(4):599; author reply 599. doi: 10.1002/ana.20084. [PMID: 15048909]
  • Dawn T Bravo, David O Harris, Stanley M Parsons. Reliable, sensitive, rapid and quantitative enzyme-based assay for gamma-hydroxybutyric acid (GHB). Journal of forensic sciences. 2004 Mar; 49(2):379-87. doi: . [PMID: 15027565]
  • Nicolas Bouché, Hillel Fromm. GABA in plants: just a metabolite?. Trends in plant science. 2004 Mar; 9(3):110-5. doi: 10.1016/j.tplants.2004.01.006. [PMID: 15003233]
  • Neil Gordon. Succinic semialdehyde dehydrogenase deficiency (SSADH) (4-hydroxybutyric aciduria, gamma-hydroxybutyric aciduria). European journal of paediatric neurology : EJPN : official journal of the European Paediatric Neurology Society. 2004; 8(5):261-5. doi: 10.1016/j.ejpn.2004.06.004. [PMID: 15341910]
  • Byung Chul Lee, Yearn Seong Choe, Dae Yoon Chi, Jin-Young Paik, Kyung-Han Lee, Yong Choi, Byung-Tae Kim. 8-cyclopentadienyltricarbonyl 99mtc 8-oxooctanoic acid: a novel radiotracer for evaluation of medium chain fatty acid metabolism in the liver. Bioconjugate chemistry. 2004 Jan; 15(1):121-7. doi: 10.1021/bc0341268. [PMID: 14733591]
  • N I Wolf, D Haas, G F Hoffmann, C Jakobs, G S Salomons, R A Wevers, U F Engelke, D Rating. Sedation with 4-hydroxybutyric acid: a potential pitfall in the diagnosis of SSADH deficiency. Journal of inherited metabolic disease. 2004; 27(2):291-3. doi: 10.1023/b:boli.0000028842.15981.6e. [PMID: 15243989]
  • Katrin Ergezinger, Reinhard Jeschke, Georg Frauendienst-Egger, Herbert Korall, K Michael Gibson, Volker H Schuster. Monitoring of 4-hydroxybutyric acid levels in body fluids during vigabatrin treatment in succinic semialdehyde dehydrogenase deficiency. Annals of neurology. 2003 Nov; 54(5):686-9. doi: 10.1002/ana.10752. [PMID: 14595661]
  • Kevin E Breitkreuz, Wendy L Allan, Owen R Van Cauwenberghe, Cornelis Jakobs, Driss Talibi, Bruno Andre, Barry J Shelp. A novel gamma-hydroxybutyrate dehydrogenase: identification and expression of an Arabidopsis cDNA and potential role under oxygen deficiency. The Journal of biological chemistry. 2003 Oct; 278(42):41552-6. doi: 10.1074/jbc.m305717200. [PMID: 12882961]
  • K Michael Gibson, Maneesh Gupta, Phillip L Pearl, Mendel Tuchman, L Gilbert Vezina, O Carter Snead, Leo M E Smit, Cornelis Jakobs. Significant behavioral disturbances in succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria). Biological psychiatry. 2003 Oct; 54(7):763-8. doi: 10.1016/s0006-3223(03)00113-6. [PMID: 14512218]
  • Toshihiro Shinka, Masafumi Ohfu, Shinichi Hirose, Tomiko Kuhara. Effect of valproic acid on the urinary metabolic profile of a patient with succinic semialdehyde dehydrogenase deficiency. Journal of chromatography. B, Analytical technologies in the biomedical and life sciences. 2003 Jul; 792(1):99-106. doi: 10.1016/s1570-0232(03)00276-9. [PMID: 12829002]
  • P L Pearl, K M Gibson, M T Acosta, L G Vezina, W H Theodore, M A Rogawski, E J Novotny, A Gropman, J A Conry, G T Berry, M Tuchman. Clinical spectrum of succinic semialdehyde dehydrogenase deficiency. Neurology. 2003 May; 60(9):1413-7. doi: 10.1212/01.wnl.0000059549.70717.80. [PMID: 12743223]
  • Alison Hinshelwood, Gail McGarvie, Elizabeth M Ellis. Substrate specificity of mouse aldo-keto reductase AKR7A5. Chemico-biological interactions. 2003 Feb; 143-144(?):263-9. doi: 10.1016/s0009-2797(02)00173-4. [PMID: 12604212]
  • Ethan Nguyen, Matthew J Picklo. Inhibition of succinic semialdehyde dehydrogenase activity by alkenal products of lipid peroxidation. Biochimica et biophysica acta. 2003 Jan; 1637(1):107-12. doi: 10.1016/s0925-4439(02)00220-x. [PMID: 12527414]
  • Phillip L Pearl, Edward J Novotny, Maria T Acosta, Cornelis Jakobs, K Michael Gibson. Succinic semialdehyde dehydrogenase deficiency in children and adults. Annals of neurology. 2003; 54 Suppl 6(?):S73-80. doi: 10.1002/ana.10629. [PMID: 12891657]
  • Myun-Ho Bang, Soo Young Choi, Tae-O Jang, Sang Kook Kim, Oh-Shin Kwon, Tae-Cheon Kang, Moo Ho Won, Jinseu Park, Nam-In Baek. Phytol, SSADH inhibitory diterpenoid of Lactuca sativa. Archives of pharmacal research. 2002 Oct; 25(5):643-6. doi: 10.1007/bf02976937. [PMID: 12433198]
  • Toshihiro Shinka, Yoshito Inoue, Morimasa Ohse, Akira Ito, Masaharu Ohfu, Shinichi Hirose, Tomiko Kuhara. Rapid and sensitive detection of urinary 4-hydroxybutyric acid and its related compounds by gas chromatography-mass spectrometry in a patient with succinic semialdehyde dehydrogenase deficiency. Journal of chromatography. B, Analytical technologies in the biomedical and life sciences. 2002 Aug; 776(1):57-63. doi: 10.1016/s1570-0232(02)00126-5. [PMID: 12127325]
  • Paola Blasi, Pietro Pilo Boyl, Mario Ledda, Andrea Novelletto, K Michael Gibson, Cornelis Jakobs, Boris Hogema, Shinjiro Akaboshi, Fabrizio Loreni, Patrizia Malaspina. Structure of human succinic semialdehyde dehydrogenase gene: identification of promoter region and alternatively processed isoforms. Molecular genetics and metabolism. 2002 Aug; 76(4):348-62. doi: 10.1016/s1096-7192(02)00105-1. [PMID: 12208142]
  • Tae-Cheon Kang, Seung-Kook Park, In-Koo Hwang, Sung-Jin An, Soo Young Choi, Sung-Woo Cho, Moo Ho Won. Spatial and temporal alterations in the GABA shunt in the gerbil hippocampus following transient ischemia. Brain research. 2002 Jul; 944(1-2):10-8. doi: 10.1016/s0006-8993(02)02596-9. [PMID: 12106661]
  • Alison Hinshelwood, Gail McGarvie, Elizabeth Ellis. Characterisation of a novel mouse liver aldo-keto reductase AKR7A5. FEBS letters. 2002 Jul; 523(1-3):213-8. doi: 10.1016/s0014-5793(02)02982-4. [PMID: 12123834]
  • Maneesh Gupta, Rachel Greven, Erwin E W Jansen, Cornelis Jakobs, Boris M Hogema, Wolfgang Froestl, O Carter Snead, Hilke Bartels, Markus Grompe, K Michael Gibson. Therapeutic intervention in mice deficient for succinate semialdehyde dehydrogenase (gamma-hydroxybutyric aciduria). The Journal of pharmacology and experimental therapeutics. 2002 Jul; 302(1):180-7. doi: 10.1124/jpet.302.1.180. [PMID: 12065715]
  • K M Gibson, D S M Schor, M Gupta, W S Guerand, H Senephansiri, T G Burlingame, H Bartels, B M Hogema, T Bottiglieri, W Froestl, O C Snead, M Grompe, C Jakobs. Focal neurometabolic alterations in mice deficient for succinate semialdehyde dehydrogenase. Journal of neurochemistry. 2002 Apr; 81(1):71-9. doi: 10.1046/j.1471-4159.2002.00784.x. [PMID: 12067239]
  • Koichi Sakurada, Masahiko Kobayashi, Hirotaro Iwase, Mineo Yoshino, Harutaka Mukoyama, Takehiko Takatori, Ken-ichi Yoshida. Production of gamma-hydroxybutyric acid in postmortem liver increases with time after death. Toxicology letters. 2002 Mar; 129(3):207-17. doi: 10.1016/s0378-4274(02)00019-x. [PMID: 11888704]
  • Peter Neu, S Seyfert, J Brockmöller, M Dettling, P Marx. Neuroleptic malignant syndrome in a patient with succinic semialdehyde dehydrogenase deficiency. Pharmacopsychiatry. 2002 Jan; 35(1):26-8. doi: 10.1055/s-2002-19830. [PMID: 11819156]
  • B M Hogema, M Gupta, H Senephansiri, T G Burlingame, M Taylor, C Jakobs, R B Schutgens, W Froestl, O C Snead, R Diaz-Arrastia, T Bottiglieri, M Grompe, K M Gibson. Pharmacologic rescue of lethal seizures in mice deficient in succinate semialdehyde dehydrogenase. Nature genetics. 2001 Oct; 29(2):212-6. doi: 10.1038/ng727. [PMID: 11544478]
  • Y Ishiguro, M Kajita, T Aoshima, K Watanabe, M Kimura, S Yamaguchi. The first case of 4-hydroxybutyric aciduria in Japan. Brain & development. 2001 Mar; 23(2):128-30. doi: 10.1016/s0387-7604(01)00181-4. [PMID: 11248463]
  • T O'connor, L S Ireland, D J Harrison, J D Hayes. Major differences exist in the function and tissue-specific expression of human aflatoxin B1 aldehyde reductase and the principal human aldo-keto reductase AKR1 family members. The Biochemical journal. 1999 Oct; 343 Pt 2(?):487-504. doi: . [PMID: 10510318]
  • E E Kaufman, T Nelson, H M Fales, D M Levin. Isolation and characterization of a hydroxyacid-oxoacid transhydrogenase from rat kidney mitochondria. The Journal of biological chemistry. 1988 Nov; 263(32):16872-9. doi: . [PMID: 3182820]
  • P P Pattarelli, W L Nyhan, K M Gibson. Oxidation of [U-14C]succinic semialdehyde in cultured human lymphoblasts: measurement of residual succinic semialdehyde dehydrogenase activity in 11 patients with 4-hydroxybutyric aciduria. Pediatric research. 1988 Oct; 24(4):455-60. doi: 10.1203/00006450-198810000-00007. [PMID: 3140205]
  • E E Kaufman, T Nelson, D Miller, N Stadlan. Oxidation of gamma-hydroxybutyrate to succinic semialdehyde by a mitochondrial pyridine nucleotide-independent enzyme. Journal of neurochemistry. 1988 Oct; 51(4):1079-84. doi: 10.1111/j.1471-4159.1988.tb03071.x. [PMID: 3138385]
  • E E Kaufman, T Nelson. Evidence for the participation of a cytosolic NADP+-dependent oxidoreductase in the catabolism of gamma-hydroxybutyrate in vivo. Journal of neurochemistry. 1987 Jun; 48(6):1935-41. doi: 10.1111/j.1471-4159.1987.tb05758.x. [PMID: 3106576]
  • G K Brown, C H Cromby, N J Manning, R J Pollitt. Urinary organic acids in succinic semialdehyde dehydrogenase deficiency: evidence of alpha-oxidation of 4-hydroxybutyric acid, interaction of succinic semialdehyde with pyruvate dehydrogenase and possible secondary inhibition of mitochondrial beta-oxidation. Journal of inherited metabolic disease. 1987; 10(4):367-75. doi: 10.1007/bf01799979. [PMID: 3126356]
  • D Rating, F Hanefeld, H Siemes, J Kneer, C Jakobs, M Hermier, P Divry. 4-Hydroxybutyric aciduria: a new inborn error of metabolism. I. Clinical review. Journal of inherited metabolic disease. 1984; 7 Suppl 1(?):90-2. doi: NULL. [PMID: 6434851]
  • C Jakobs, J Kneer, D Rating, F Hanefeld, P Divry, M Hermier. 4-Hydroxybutyric aciduria: a new inborn error of metabolism. II. Biochemical findings. Journal of inherited metabolic disease. 1984; 7 Suppl 1(?):92-4. doi: NULL. [PMID: 6434852]
  • K M Gibson, L Sweetman. Enzymatic preparation of radiolabeled succinic semialdehyde. Analytical biochemistry. 1983 Dec; 135(2):436-7. doi: 10.1016/0003-2697(83)90707-8. [PMID: 6660517]