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phenylalanine metabolism

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https://www.readbyqxmd.com/read/29457782/hypothalamic-er-associated-degradation-regulates-pomc-maturation-feeding-and-age-associated-obesity
#1
Geun Hyang Kim, Guojun Shi, Diane Rm Somlo, Leena Haataja, Soobin Song, Qiaoming Long, Eduardo A Nillni, Malcolm J Low, Peter Arvan, Martin G Myers, Ling Qi
Pro-opiomelanocortin (POMC) neurons function as key regulators of metabolism and physiology by releasing prohormone-derived neuropeptides with distinct biological activities. However, our understanding of early events in prohormone maturation in the ER remains incomplete. Highlighting the significance of this gap in knowledge, a single POMC cysteine-to-phenylalanine mutation at position 28 (POMC-C28F) is defective for ER processing and causes early onset obesity in a dominant-negative manner in humans through an unclear mechanism...
February 19, 2018: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29454221/-13-c-phenylalanine-breath-test-and-serum-biopterin-in-schizophrenia-bipolar-disorder-and-major-depressive-disorder
#2
Toshiya Teraishi, Masahiro Kajiwara, Hiroaki Hori, Daimei Sasayama, Shinsuke Hidese, Junko Matsuo, Ikki Ishida, Yasuhiro Kajiwara, Yuji Ozeki, Miho Ota, Kotaro Hattori, Teruhiko Higuchi, Hiroshi Kunugi
Phenylalanine is required for the synthesis of the neurotransmitters dopamine, noradrenaline, and adrenaline. The rate-limiting step for phenylalanine metabolism is catalyzed by phenylalanine hydroxylase (PAH) and its cofactor tetrahydrobiopterin. We aimed to detect altered phenylalanine metabolism in major psychiatric disorders using the l-[1-13 C]phenylalanine breath test (13 C-PBT) and serum biopterin levels. We also investigated association of PAH mutations with schizophrenia and phenylalanine metabolism...
February 3, 2018: Journal of Psychiatric Research
https://www.readbyqxmd.com/read/29454001/brain-bioenergetics-in-rats-with-acute-hyperphenylalaninemia
#3
Nádia Weber Dimer, Bruna Klippel Ferreira, Jotele Fontana Agostini, Maria Luiza Gomes, Luiza Wilges Kist, Fernanda Malgarin, Milena Carvalho-Silva, Lara Mezari Gomes, Joyce Rebelo, Marisa Jádna Silva Frederico, Fátima Regina Mena Barreto Silva, Eduardo Pacheco Rico, Mauricio Reis Bogo, Emilio Luiz Streck, Gustavo Costa Ferreira, Patrícia Fernanda Schuck
Phenylketonuria (PKU) is a disorder of phenylalanine (Phe) metabolism caused by deficient phenylalanine hydroxylase activity. The deficiency results in increased levels of Phe and its metabolites in fluids and tissues of patients. PKU patients present neurological signals and symptoms including hypomyelination and intellectual deficit. This study assessed brain bioenergetics at 1 h after acute Phe administration to induce hyperphenylalaninemia (HPA) in rats. Wistar rats were randomized in two groups: HPA animals received a single subcutaneous administration of Phe (5...
February 14, 2018: Neurochemistry International
https://www.readbyqxmd.com/read/29448137/differential-metabolism-of-l-phenylalanine-in-the-formation-of-aromatic-volatiles-in-melon-cucumis-melo-l-fruit
#4
Itay Gonda, Rachel Davidovich-Rikanati, Einat Bar, Shery Lev, Pliaa Jhirad, Yuval Meshulam, Guy Wissotsky, Vitaly Portnoy, Joseph Burger, Arthur A Schaffer, Yaakov Tadmor, James J Giovannoni, Zhangjun Fei, Aaron Fait, Nurit Katzir, Efraim Lewinsohn
Studies on the active pathways and the genes involved in the biosynthesis of L-phenylalanine-derived volatiles in fleshy fruits are sparse. Melon fruit rinds converted stable-isotope labeled L-phe into more than 20 volatiles. Phenylpropanes, phenylpropenes and benzenoids are apparently produced via the well-known phenylpropanoid pathway involving phenylalanine ammonia lyase (PAL) and being (E)-cinnamic acid a key intermediate. Phenethyl derivatives seemed to be derived from L-phe via a separate biosynthetic route not involving (E)-cinnamic acid and PAL...
February 12, 2018: Phytochemistry
https://www.readbyqxmd.com/read/29444251/cultured-equine-satellite-cells-as-a-model-system-to-assess-leucine-stimulated-protein-synthesis-in-horse-muscle
#5
M L DeBoer, K M Martinson, M S Pampusch, A M Hansen, S M Wells, C Ward, M Hathaway
Leucine has been shown to stimulate the mammalian/mechanistic target of rapamycin (mTOR) signaling pathway which plays numerous key regulatory roles in cell growth, survival and metabolism including protein synthesis in a number of species. However, previous work with equine satellite cells has suggested distinct species differences in regards to physiological effects and the magnitude of responses to growth factors and regulators. Because there is limited research available regarding the role of leucine in regulating equine skeletal muscle protein synthesis, the objective of this study was to evaluate the effect of leucine on the mTOR signaling pathway in cultured equine satellite...
February 12, 2018: Journal of Animal Science
https://www.readbyqxmd.com/read/29442259/lc-ms-based-serum-metabolomics-reveals-a-distinctive-signature-in-patients-with-rheumatoid-arthritis
#6
Ju Li, Nan Che, Lingxiao Xu, Qian Zhang, Qi Wang, Wenfeng Tan, Miaojia Zhang
Metabolomics has been applied to explore altered metabolite profiles in disease and identify unique metabolic signatures in recent years. We aim to characterize the metabolic profile of rheumatoid arthritis patients and explore its underlying pathological processes using metabolomics approach. Serum samples from 30 rheumatoid arthritis (RA) patients, 30 primary Sjogren's syndrome (pSS) patients, and 32 healthy controls (HC) were collected. The sample was analyzed by ultra-high-performance liquid chromatography coupled with high-resolution mass spectrometry (UPLC-HRMS)...
February 13, 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/29438896/transcriptomic-profiling-reveals-pigment-regulation-during-peanut-testa-development
#7
Liyun Wan, Bei Li, Yong Lei, Liying Yan, Dongxin Huai, Yanping Kang, Huifang Jiang, Jiazhuang Tan, Boshou Liao
Although peanut (Arachis hypogaea L.) is one of the most important edible oil crops globally, pigments present in the testa influence both the processing efficiency and the quality of the oil. In peanut, polymeric phenolic compounds are present in the episperm rather than in the endothelium and their levels increase during ripening; therefore, to better understand testa development, and especially the accumulation of pigments, RNA-Seq was applied to elucidate the mechanisms underlying the regulation of peanut testae at three different developmental stages (i...
February 2, 2018: Plant Physiology and Biochemistry: PPB
https://www.readbyqxmd.com/read/29436067/identification-and-functional-analyses-of-differentially-expressed-metabolites-in-early-stage-endometrial-carcinoma
#8
Kun Shi, Qiong Wang, Yao Su, Xingcui Xuan, Yaqiong Liu, Weiwei Chen, Yuanmin Qian, Gendie E Lash
Diagnosis of endometrial cancer is primarily based on symptoms and imaging, with early-stage disease being difficult to diagnose. Therefore, development of potential diagnostic biomarkers is required. Metabolomics, a quantitative measurement of the dynamic metabolism in living systems, can be applied to determine metabolite profiles in different disease states. Here, serum metabolomics was performed in forty-six early stage endometrial cancer patients and forty-six healthy volunteers. In addition, the effect of identified metabolites on tumour cell behavior (invasion, migration, proliferation, apoptosis, autophagy), was examined in endometrial cancer cell lines...
February 12, 2018: Cancer Science
https://www.readbyqxmd.com/read/29435916/the-metabolic-disturbances-of-motoneurons-exposed-to-glutamate
#9
Blandine Madji Hounoum, Hélène Blasco, Emmanuelle Coque, Patrick Vourc'h, Patrick Emond, Philippe Corcia, Christian R Andres, Cédric Raoul, Sylvie Mavel
Glutamate-induced excitotoxicity is considered as one of the major pathophysiological factors of motoneuron death in amyotrophic lateral sclerosis and other motoneuron diseases. In order to expand our knowledge on mechanisms of glutamate-induced excitotoxicity, the present study proposes to determine the metabolic consequences of glutamate and astrocytes in primary enriched motoneuron culture. Using liquid chromatography coupled to high-resolution mass spectrometry (LC-HRMS), we showed that the presence of astrocytes and glutamate profoundly modified the metabolic profile of motoneurons...
February 12, 2018: Molecular Neurobiology
https://www.readbyqxmd.com/read/29432026/developmental-trajectories-of-executive-and-verbal-processes-in-children-with-phenylketonuria
#10
Zoë W Hawks, Michael J Strube, Neco X Johnson, Dorothy K Grange, Desirée A White
Phenylketonuria (PKU) is a hereditary disorder characterized by disrupted phenylalanine metabolism and cognitive impairment. However, the precise nature and developmental trajectory of this cognitive impairment remains unclear. The present study used a verbal fluency task to dissociate executive and verbal processes in children with PKU (n = 23; 7-18 years) and controls (n = 44; 7-19 years). Data were collected at three longitudinal timepoints over a three-year period, and the contributions of age, group, and their interaction to fluency performance were evaluated...
February 12, 2018: Developmental Neuropsychology
https://www.readbyqxmd.com/read/29416559/a-systematic-optimization-of-styrene-biosynthesis-in-escherichia-coli-bl21-de3
#11
Changqing Liu, Xiao Men, Hailin Chen, Meijie Li, Zhaorui Ding, Guoqiang Chen, Fan Wang, Haobao Liu, Qian Wang, Youshuang Zhu, Haibo Zhang, Mo Xian
Background: Styrene is a versatile commodity petrochemical used as a monomer building-block for the synthesis of many useful polymers. Although achievements have been made on styrene biosynthesis in microorganisms, several bottleneck problems limit factors for further improvement in styrene production. Results: A two-step styrene biosynthesis pathway was developed and introduced into Escherichia coli BL21(DE3). Systematic optimization of styrene biosynthesis, such as enzyme screening, codon and plasmid optimization, metabolic flow balance, and in situ fermentation was performed...
2018: Biotechnology for Biofuels
https://www.readbyqxmd.com/read/29413978/metabolomics-analysis-of-serum-reveals-the-effect-of-danggui-buxue-tang-on-fatigued-mice-induced-by-exhausting-physical-exercise
#12
Xiaoyao Miao, Bingkun Xiao, Sufang Shui, Jianyun Yang, Rongqing Huang, Junxing Dong
Danggui Buxue Tang (DBT), believed to invigorate 'Qi' (vital energy) and nourish 'Blood' (body circulation), is a traditional Chinese medicine formula. In this study, a metabolomics approach with gas chromatography coupled to mass spectrometry combined with pattern recognition was adopted to investigate the underlying mechanism of the antifatigue effect of DBT on fatigue of mice induced by weight-loaded forced swimming. Fourteen endogenous metabolites, up-regulated or down-regulated, were identified in the model mice by analysis tools of partial least-squares discriminant analysis (PLS-DA) and XCMS online software...
January 26, 2018: Journal of Pharmaceutical and Biomedical Analysis
https://www.readbyqxmd.com/read/29413232/pah-mutation-spectrum-and-correlation-with-pku-manifestation-in-north-jiangsu-province-population
#13
Zhen-Wen Wang, Shi-Wen Jiang, Bao-Cheng Zhou
Phenylketonuria (PKU) is a common autosomal recessive disorder of phenylalanine metabolism and mainly results a deficiency of phenylalanine hydroxylase gene (PAH). The incidence of various PAH mutations have race and ethnicity differences. We report a spectrum of PAH mutations complied from 35 PKU children who are all Chinese Han population from north Jiangsu in this study. All 13 exons and their flanking intron sequences of PAH were determined by Ion Torrent PGM™ sequencing. The relationship of genotype and phenotype was analyzed based on the sum of the arbitrary value (AV) values of the two alleles...
February 2018: Kaohsiung Journal of Medical Sciences
https://www.readbyqxmd.com/read/29404866/differential-expression-of-the-pal-gene-family-in-rice-seedlings-exposed-to-chromium-by-microarray-analysis
#14
Xiao-Zhang Yu, Wei-Jia Fan, Yu-Juan Lin, Fei-Fei Zhang, Dharmendra K Gupta
Phenylalanine ammonia-lyase (PAL) is one of the principle enzymes involved in plant's secondary metabolism. Expression of individual isogene from the PAL gene family is variable with species of plants in responses to different stresses. In this study, transcriptome analysis of the PAL gene family in rice seedlings exposed to potassium chromate Cr(VI) or chromium nitrate Cr(III) was conducted using Agilent 44K rice microarray and real-time quantitative RT-PCR. Uptake and accumulation of both Cr species by rice seedlings and their effect on PAL activity were also determined...
February 5, 2018: Ecotoxicology
https://www.readbyqxmd.com/read/29394889/comparative-transcriptomic-analysis-of-resistant-and-susceptible-alfalfa-cultivars-medicago-sativa-l-after-thrips-infestation
#15
Xiongbing Tu, Zhongkuan Liu, Zehua Zhang
BACKGROUND: Plant breeding for resistance to agricultural pests is an essential element in the development of integrated crop management systems; however, the molecular and genetic mechanisms underlying resistance are poorly understood. In this pilot study, a transcriptomic analysis of a resistant (R) vs. a susceptible (S) variety of alfalfa, with (+T) or without (-T) thrips (= 4 treatments) was conducted, 'GN-1' (China) was defined as the resistant cultivar, and 'WL323' (America) was defined as the susceptible cultivar...
February 2, 2018: BMC Genomics
https://www.readbyqxmd.com/read/29394372/jasmonate-responsive-myb-factors-spatially-repress-rutin-biosynthesis-in-fagopyrum-tataricum
#16
Kaixuan Zhang, Maria D Logacheva, Yu Meng, Jianping Hu, Dongpu Wan, Long Li, Janovská Dagmar, Zhiyong Wang, Milen I Georgiev, Zhuo Yu, Fuyu Yang, Mingli Yan, Meiliang Zhou
Jasmonates (JAs) are plant hormones that induce many secondary metabolites accumulation, such as rutin in buckwheat, via regulation of JAs-responsive transcription factors (TFs). Here, we report on the identification of a clade of JAs-responsive subgroup 4 MYB TFs, FtMYB13, FtMYB14, FtMYB15 and FtMYB16, directly repressing rutin biosynthesis in Fagopyrum tataricum. Immunoblot analysis showed that FtMYB13, FtMYB14 and FtMYB15 could be degraded via 26S proteasome in COI1-dependent JA signaling pathway, and this degradation is due to the SID motif in their C-terminus...
January 31, 2018: Journal of Experimental Botany
https://www.readbyqxmd.com/read/29390883/mutational-spectrum-of-the-phenylalanine-hydroxylase-gene-in-patients-with-phenylketonuria-in-the-central-region-of-china
#17
Zhan Zhang, Jun-Jun Gao, Yang Feng, Lin-Lin Zhu, Huan Yan, Xu-Feng Shi, Ai-Min Chang, Ying Shi, Ping Wang
Phenylketonuria (PKU, OMIM 261600) caused by phenylalanine hydroxylase (PAH) deficiency is an autosomal recessive disease that is characterized by abnormalities of phenylalanine metabolism. In this study, a total of 77 patients, originating from the central region of China and who were diagnosed with PAH deficiency at the third affiliated hospital of Zhengzhou University, were enrolled in this study. The 13 exons and 12 flanking introns of the PAH gene were analyzed by Sanger sequencing and next generation sequencing...
February 1, 2018: Scandinavian Journal of Clinical and Laboratory Investigation
https://www.readbyqxmd.com/read/29389572/metabolic-profiling-investigation-of-fritillaria-thunbergii-miq-by-gas-chromatography-mass-spectrometry
#18
Ming-Chao Cui, Shao-Jun Chen, Han-Hua Wang, Zhao-Hui Li, Hong-Jiang Chen, Yong Chen, Hai-Bin Zhou, Xiang Li, Jian-Wei Chen
Thunberg fritillary bulb (the dry bulbs of Fritillaria thunbergii Miq.), a traditional Chinese Medicine, is widely applied as an expectorant and antitussive. In this investigation, the primary metabolites of bulbs, flowers, leaves, and stems of F. thunbergii were analyzed by gas chromatography-mass spectrometry. Principal component analysis, partial least squares-discriminate analysis, orthogonal projection to latent structures-discriminate analysis, and heat map analysis showed that there were dissimilar metabolites, and a negative correlation between amino acids and saccharides in different analytes...
January 2018: Journal of Food and Drug Analysis
https://www.readbyqxmd.com/read/29388394/composition-of-gut-microbiota-in-obese-and-normal-weight-mexican-school-age-children-and-its-association-with-metabolic-traits
#19
B E López-Contreras, S Morán-Ramos, R Villarruel-Vázquez, L Macías-Kauffer, H Villamil-Ramírez, P León-Mimila, J Vega-Badillo, F Sánchez-Muñoz, L E Llanos-Moreno, A Canizalez-Román, B Del Río-Navarro, I Ibarra-González, M Vela-Amieva, T Villarreal-Molina, A Ochoa-Leyva, C A Aguilar-Salinas, S Canizales-Quinteros
BACKGROUND: Childhood obesity is a serious public health problem in Mexico. Adult gut microbiota composition has been linked to obesity, but few studies have addressed the role of gut microbiota in childhood obesity. OBJECTIVES: The aim of this study is to compare gut microbiota composition in obese and normal-weight children and to associate gut microbiota profiles with amino acid serum levels and obesity-related metabolic traits. METHODS: Microbial taxa relative abundance was determined by 16S rRNA sequencing in 67 normal-weight and 71 obese children aged 6-12 years...
December 5, 2017: Pediatric Obesity
https://www.readbyqxmd.com/read/29382315/genetic-engineering-of-escherichia-coli-to-improve-l-phenylalanine-production
#20
Yongfei Liu, Yiran Xu, Dongqin Ding, Jianping Wen, Beiwei Zhu, Dawei Zhang
BACKGROUND: L-phenylalanine (L-Phe) is an essential amino acid for mammals and applications expand into human health and nutritional products. In this study, a system level engineering was conducted to enhance L-Phe biosynthesis in Escherichia coli. RESULTS: We inactivated the PTS system and recruited glucose uptake via combinatorial modulation of galP and glk to increase PEP supply in the Xllp01 strain. In addition, the HTH domain of the transcription factor TyrR was engineered to decrease the repression on the transcriptional levels of L-Phe pathway enzymes...
January 30, 2018: BMC Biotechnology
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