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Electron-transfer-flavoprotein, alpha polypeptide

ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008] (from NCBI)
Top mentioned proteins: ACID, HAD, CAN, N-acetyl-beta-D-glucosaminidase, AGE
Papers on GA2
Breadfruit (Artocarpus altilis) gibberellin 2-oxidase genes in stem elongation and abiotic stress response.
Underhill et al., Australia. In Plant Physiol Biochem, Jan 2016
As a first step toward understanding the molecular mechanism of growth regulation in the species, we isolated a cohort of four full-length GA2-oxidase cDNAs, AaGA2ox1- AaGA2ox4 from breadfruit.
The regulation of ER export and Golgi retention of ST3Gal5 (GM3/GM4 synthase) and B4GalNAcT1 (GM2/GD2/GA2 synthase) by arginine/lysine-based motif adjacent to the transmembrane domain.
Inokuchi et al., Sendai, Japan. In Glycobiology, Dec 2015
In the Golgi maturation model, the Golgi cisternae dynamically mature along a secretory pathway.
GM1 Ganglioside is Involved in Epigenetic Activation Loci of Neuronal Cells.
Yu et al., Augusta, United States. In Neurochem Res, Nov 2015
We previously demonstrated that acetylation of histones H3 and H4 on the N-acetylgalactosaminyltransferase I (GalNAcT, GA2/GM2/GD2/GT2-synthase) gene promoter resulted in recruitment of trans-activation factors.
Clinical, biochemical and molecular investigation of adult-onset glutaric acidemia type II: Characteristics in comparison with pediatric cases.
Yamaguchi et al., Izumo, Japan. In Brain Dev, Oct 2015
In this study, clinical, biochemical, and molecular characteristics of 2 Japanese patients with adult-onset glutaric acidemia type II (GA2) were investigated and compared with those of pediatric cases.
Conservation of G-Protein Epitopes in Respiratory Syncytial Virus (Group A) Despite Broad Genetic Diversity: Is Antibody Selection Involved in Virus Evolution?
Melero et al., Madrid, Spain. In J Virol, Aug 2015
Multiple genotypes cocirculated during prolonged periods of time, but recent dominance of the GA2 genotype was noticed in several studies, and it is highlighted here with sequences from viruses circulating recently in Spain and Panama.
Synthesis, characterization and analysis of the acrylamide- and glycidamide-glutathione conjugates.
Wu et al., Taipei, Taiwan. In Chem Biol Interact, Aug 2015
AA and GA can be detoxified by glutathione-S-transferase to form AA and isomeric GA glutathione conjugates (AA-, GA2- and GA3-GSH, respectively), which can be further metabolized to mercapturic acids (MAs).
Prevalence of human respiratory syncytial virus circulating in Iran.
Mokhtari-Azad et al., Tehrān, Iran. In J Infect Public Health, Aug 2015
Phylogenetic studies showed that genotypes GA1, GA2, GA5, and BA co-circulated in Iran in 2007-2013.
[Epidemiologic characteristics and the relationship with disease severity of respiratory syncytial virus genotypes from children with lower respiratory tract infection in the southern Zhejiang province].
Lin et al., Wenzhou, China. In Zhonghua Er Ke Za Zhi, Jul 2015
A total of 112 strains of complete sequences of G genes were obtained, including four subtype A genotypes NA1, NA4, GA2 and ON1, and six subtype B genotypes BA8-10, BA-C, CB1, and GB2.
Genome-wide transcriptomic profiling of ramie (Boehmeria nivea L. Gaud) in response to cadmium stress.
Tang et al., Changsha, China. In Gene, Apr 2015
Among these 16 DEGs, 2 genes encoding GA2-oxidase (a major enzyme for deactivating bioactive gibberellins [GAs]) showed markedly up-regulated expression in Cd stressed ramie.
AAV-mediated gene delivery in a feline model of Sandhoff disease corrects lysosomal storage in the central nervous system.
Martin et al., Boston, United States. In Asn Neuro, Mar 2015
SD presents with an accumulation of GM2 and its asialo derivative GA2, primarily in the central nervous system.
Association between ETFA genotype and activity of superoxide dismutase, catalase and glutathione peroxidase in cryopreserved sperm of Holstein-Friesian bulls.
Kamiński et al., Olsztyn, Poland. In Reprod Domest Anim, Feb 2015
The aim of this study was to determine whether C/T missense mutation within the ETFA gene is associated with sperm antioxidant enzymatic activity.
Sequencing and analysis of globally obtained human respiratory syncytial virus A and B genomes.
Henrickson et al., Milwaukee, United States. In Plos One, 2014
We found multiple clades co-circulating globally for both RSV A and B. The predominant clades were GA2 and GA5 for RSVA and BA for RSVB.
Update on clinical aspects and treatment of selected vitamin-responsive disorders II (riboflavin and CoQ 10).
Horvath, Newcastle upon Tyne, United Kingdom. In J Inherit Metab Dis, 2012
Mutations in the electron tranferring fravoprotein genes (ETFA/ETFB) and its dehydrogenase (ETFDH) are causative for multiple acyl-CoA dehydrogenase deficiency.
Electron transfer flavoprotein domain II orientation monitored using double electron-electron resonance between an enzymatically reduced, native FAD cofactor, and spin labels.
Eaton et al., Denver, United States. In Protein Sci, 2011
These results are consistent with the electron transfer flavoprotein alpha II domain adopting orientations in solution that deviate from the crystal structure of free ETF towards the active, substrate-bound orientation.
A polymorphic position in electron transfer flavoprotein modulates kinetic stability as evidenced by thermal stress.
Gomes et al., Lisbon, Portugal. In Febs Lett, 2011
investigations are compatible with the notion that the ETFalpha-T171 variant displays an altered conformational landscape that results in reduced protein function under thermal stress
Mutational hotspots in electron transfer flavoprotein underlie defective folding and function in multiple acyl-CoA dehydrogenase deficiency.
Gomes et al., Lisbon, Portugal. In Biochim Biophys Acta, 2010
Data established structural hotspots within the ETF fold, and provided a rationale for the prediction of effects of mutations in ETF.
Transient multiple acyl-CoA dehydrogenation deficiency in a newborn female caused by maternal riboflavin deficiency.
Christodoulou et al., Sydney, Australia. In Mol Genet Metab, 2007
No mutations in electron-transfer-flavoprotein but maternal riboflavin deficiency led to multiple acyl-CoA dehydrogenation deficiency
Electron transfer flavoprotein deficiency: functional and molecular aspects.
Vianey-Saban et al., Lyon, France. In Mol Genet Metab, 2006
Tissue samples from 16 unrelated patients with ETF deficiency were analysed and the majority of the patients had mutations in the ETFA gene.
Beta1,4-N-acetylgalactosaminyltransferase--GM2/GD2 synthase: a key enzyme to control the synthesis of brain-enriched complex gangliosides.
Furukawa et al., Nagoya, Japan. In Biochim Biophys Acta, 2003
Beta1,4-N-acetylgalactosaminyltransferase (GM2/GD2 synthase) is a key enzyme which catalyzes the conversion of GM3, GD3 and lactosylceramide (LacCer) to GM2, GD2 and asialo-GM2 (GA2), respectively.
Oligosaccharide specificity of galectins: a search by frontal affinity chromatography.
Kasai et al., Japan. In Biochim Biophys Acta, 2002
As a matter of fact, no galectin could bind to glycolipid-type glycans (e.g., GM2, GA2, Gb3), complex-type N-glycans, of which both 6-OH groups are sialylated, nor Le-related antigens (e.g., Le(x), Le(a)).
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