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Article ; Online: N

Lalevée, Jacques / Tehfe, Mohamad-Ali / Zein-Fakih, Abbas / Ball, Bruno / Telitel, Sofia / Morlet-Savary, Fabrice / Graff, Bernadette / Fouassier, Jean Pierre

ACS macro letters

2012 Volume 1, Issue 7, Page(s) 802–806

Abstract: N ...

Abstract	N
Language	English
Publishing date	2012-06-11
Publishing country	United States
Document type	Journal Article
ISSN	2161-1653
ISSN (online)	2161-1653
DOI	10.1021/mz3002325
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Article ; Online: Oral cysteamine bitartrate and N-acetylcysteine for patients with infantile neuronal ceroid lipofuscinosis: a pilot study.

Levin, Sondra W / Baker, Eva H / Zein, Wadih M / Zhang, Zhongjian / Quezado, Zenaide M N / Miao, Ning / Gropman, Andrea / Griffin, Kurt J / Bianconi, Simona / Chandra, Goutam / Khan, Omar I / Caruso, Rafael C / Liu, Aiyi / Mukherjee, Anil B

The Lancet. Neurology

2014 Volume 13, Issue 8, Page(s) 777–787

Abstract: ... We have previously reported that phosphocysteamine and N-acetylcysteine mediate ceroid depletion ... cysteamine bitartrate and N-acetylcysteine is beneficial for patients with neuronal ceroid lipofuscinosis.: Methods ... recruited from physician referrals. Patients received oral cysteamine bitartrate (60 mg/kg per day) and N ...

Abstract	Background: Infantile neuronal ceroid lipofuscinosis is a devastating neurodegenerative lysosomal storage disease caused by mutations in the gene (CLN1 or PPT1) encoding palmitoyl-protein thioesterase-1 (PPT1). We have previously reported that phosphocysteamine and N-acetylcysteine mediate ceroid depletion in cultured cells from patients with this disease. We aimed to assess whether combination of oral cysteamine bitartrate and N-acetylcysteine is beneficial for patients with neuronal ceroid lipofuscinosis. Methods: Children between 6 months and 3 years of age with infantile neuronal ceroid lipofuscinosis with any two of the seven most lethal PPT1 mutations were eligible for inclusion in this pilot study. All patients were recruited from physician referrals. Patients received oral cysteamine bitartrate (60 mg/kg per day) and N-acetylcysteine (60 mg/kg per day) and were assessed every 6-12 months until they had an isoelectric electroencephalogram (EEG, attesting to a vegetative state) or were too ill to travel. Patients were also assessed by electroretinography, brain MRI and magnetic resonance spectroscopy (MRS), and electron microscopic analyses of leukocytes for granular osmiophilic deposits (GRODs). Children also underwent physical and neurodevelopmental assessments on the Denver scale. Outcomes were compared with the reported natural history of infantile neuronal ceroid lipofuscinosis and that of affected older siblings. This trial is registered with ClinicalTrials.gov, number NCT00028262. Findings: Between March 14, 2001, and June 30, 2012, we recruited ten children with infantile neuronal ceroid lipofuscinosis; one child was lost to follow-up after the first visit and nine patients (five girls and four boys) were followed up for 8 to 75 months. MRI showed abnormalities similar to those in previous reports; brain volume and N-acetyl aspartic acid (NAA) decreased steadily, but no published quantitative MRI or MRS studies were available for comparison. None of the children acquired new developmental skills, and their retinal function decreased progressively. Average time to isoelectric EEG (52 months, SD 13) was longer than reported previously (36 months). At the first follow-up visit, peripheral leukocytes in all nine patients showed virtually complete depletion of GRODs. Parents and physicians reported less irritability, improved alertness, or both in seven patients. No treatment-related adverse events occurred apart from mild gastrointestinal discomfort in two patients, which disappeared when liquid cysteamine bitartrate was replaced with capsules. Interpretation: Our findings suggest that combination therapy with cysteamine bitartrate and N-acetylcysteine is associated with delay of isoelectric EEG, depletion of GRODs, and subjective benefits as reported by parents and physicians. Our systematic and quantitative report of the natural history of patients with infantile neuronal ceroid lipofuscinosis provides a guide for future assessment of experimental therapies. Funding: National Institutes of Health.
MeSH term(s)	Acetylcysteine/administration & dosage ; Administration, Oral ; Child, Preschool ; Cysteamine/administration & dosage ; Drug Therapy, Combination ; Electroencephalography/methods ; Female ; Follow-Up Studies ; Humans ; Infant ; Male ; Neuronal Ceroid-Lipofuscinoses/diagnosis ; Neuronal Ceroid-Lipofuscinoses/drug therapy ; Neuronal Ceroid-Lipofuscinoses/physiopathology ; Pilot Projects
Chemical Substances	Cysteamine (5UX2SD1KE2) ; Acetylcysteine (WYQ7N0BPYC)
Language	English
Publishing date	2014-07-02
Publishing country	England
Document type	Clinical Trial ; Journal Article ; Research Support, N.I.H., Extramural ; Research Support, N.I.H., Intramural
ZDB-ID	2081241-3
ISSN	1474-4465 ; 1474-4422
ISSN (online)	1474-4465
ISSN	1474-4422
DOI	10.1016/S1474-4422(14)70142-5
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Article ; Online: The type II integral ER membrane protein VAP-B homolog in C. elegans is cleaved to release the N-terminal MSP domain to signal non-cell-autonomously.

Zein-Sabatto, Hala / Cole, Tim / Hoang, Hieu D / Tiwary, Ekta / Chang, Chenbei / Miller, Michael A

Developmental biology

2020 Volume 470, Page(s) 10–20

Abstract: VAMP/synaptobrevin-associated protein B (VAP-B) is a type II ER membrane protein, but its N ... specific expression of an N-terminally FLAG-tagged VPR-1 rescues underdeveloped gonad and sterility defects ... 1 is present at the distal gonad. Mass spectrometry analysis of a smaller product of the N ...

Abstract	VAMP/synaptobrevin-associated protein B (VAP-B) is a type II ER membrane protein, but its N-terminal MSP domain (MSPd) can be cleaved and secreted. Mutations preventing the cleavage and secretion of MSPd have been implicated in cases of human neurodegenerative diseases. The site of VAP cleavage and the tissues capable in releasing the processed MSPd are not understood. In this study, we analyze the C. elegans VAP-B homolog, VPR-1, for its processing and secretion from the intestine. We show that intestine-specific expression of an N-terminally FLAG-tagged VPR-1 rescues underdeveloped gonad and sterility defects in vpr-1 null hermaphrodites. Immunofluorescence studies reveal that the tagged intestinal expressed VPR-1 is present at the distal gonad. Mass spectrometry analysis of a smaller product of the N-terminally tagged VPR-1 identifies a specific cleavage site at Leu156. Mutation of the leucine results in loss of gonadal MSPd signal and reduced activity of the mutant VPR-1. Thus, we report for the first time the cleavage site of VPR-1 and provide direct evidence that intestinally expressed VPR-1 can be released and signal in the distal gonad. These results establish the foundation for further exploration of VAP cleavage, MSPd secretion, and non-cell-autonomous signaling in development and diseases.
MeSH term(s)	Animals ; Animals, Genetically Modified ; Caenorhabditis elegans/embryology ; Caenorhabditis elegans/genetics ; Caenorhabditis elegans/growth & development ; Caenorhabditis elegans/metabolism ; Caenorhabditis elegans Proteins/chemistry ; Caenorhabditis elegans Proteins/genetics ; Caenorhabditis elegans Proteins/metabolism ; Endoplasmic Reticulum/metabolism ; Genes, Helminth ; Gonads/chemistry ; Gonads/growth & development ; Gonads/metabolism ; Helminth Proteins/chemistry ; Helminth Proteins/metabolism ; Hermaphroditic Organisms/genetics ; Hermaphroditic Organisms/metabolism ; Hermaphroditic Organisms/physiology ; Infertility ; Intestines/cytology ; Intestines/physiology ; Leucine/metabolism ; Membrane Proteins/chemistry ; Membrane Proteins/genetics ; Membrane Proteins/metabolism ; Phenotype ; Point Mutation ; Protein Domains ; Protein Processing, Post-Translational
Chemical Substances	Caenorhabditis elegans Proteins ; Helminth Proteins ; Membrane Proteins ; VPR-1 protein, C elegans ; major sperm protein, nematode ; Leucine (GMW67QNF9C)
Language	English
Publishing date	2020-11-05
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	1114-9
ISSN	1095-564X ; 0012-1606
ISSN (online)	1095-564X
ISSN	0012-1606
DOI	10.1016/j.ydbio.2020.10.015
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Article ; Online: Seasonal changes of C and N non-structural compounds in the stem sapwood of adult sessile oak and beech trees.

El Zein, R / Maillard, P / Bréda, N / Marchand, J / Montpied, P / Gérant, D

Tree physiology

2011 Volume 31, Issue 8, Page(s) 843–854

Abstract: ... N) cycling of the stem rather than in N storage and remobilization to the other growing organs ...

Abstract	We assessed the pools of non-structural nitrogen compounds (NSNC) through a year, thereby addressing the question of whether mature sessile oak [Quercus petraea (Matt.) Liebl.] and beech (Fagus sylvatica L.), which differ in wood anatomy and growth patterns, exhibit contrasting seasonal dynamics of NSNC pools as previously shown for non-structural carbohydrate (NSC) pools. Seasonal fluctuations of NSNC (amino acids and soluble proteins) and NSC (starch and soluble sugars) pools were analyzed in the inner and the outer stem sapwood. In oak, NSC showed marked seasonal variation within the stem sapwood (accumulation during winter and decrease during bud burst and early wood growth), whereas in beech seasonal fluctuations in NSC were of minor amplitude. Even if the distribution and intensity of the NSNC pools differed between the two species, NSNC of the stem sapwood did not show seasonal variation. The most significant change in NSNC pools was the seasonal fluctuation of protein composition. In both species, two polypeptides of 13 kDa (PP13) and 26 kDa (PP26) accumulated during the coldest period in parallel with starch to sugar conversion and disappeared with the onset of spring growth. The absence of seasonal changes in total soluble protein concentration suggests that the polypeptides are involved in the internal nitrogen (N) cycling of the stem rather than in N storage and remobilization to the other growing organs of the tree.
MeSH term(s)	Carbohydrate Metabolism ; Fagus/growth & development ; Fagus/metabolism ; Nitrogen Compounds/metabolism ; Plant Stems/growth & development ; Quercus/growth & development ; Quercus/metabolism ; Seasons ; Temperature
Chemical Substances	Nitrogen Compounds
Language	English
Publishing date	2011-08
Publishing country	Canada
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	743341-4
ISSN	1758-4469 ; 0829-318X
ISSN (online)	1758-4469
ISSN	0829-318X
DOI	10.1093/treephys/tpr074
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Article ; Online: Nitrogen sources for current-year shoot growth in 50-year-old sessile oak trees: an in situ (15)N labeling approach.

El Zein, Rana / Bréda, Nathalie / Gérant, Dominique / Zeller, Bernd / Maillard, P

Tree physiology

2011 Volume 31, Issue 12, Page(s) 1390–1400

Abstract: We used long-term in situ (15)N labeling of the soil to investigate the contribution of the two ... main nitrogen (N) sources (N uptake versus N reserves) to sun shoot growth from bud burst to full leaf ... expansion in 50-year-old sessile oaks. Recovery of (15)N by growing compartments (leaves, twigs and buds ...

Abstract	We used long-term in situ (15)N labeling of the soil to investigate the contribution of the two main nitrogen (N) sources (N uptake versus N reserves) to sun shoot growth from bud burst to full leaf expansion in 50-year-old sessile oaks. Recovery of (15)N by growing compartments (leaves, twigs and buds) and presence of (15)N in phloem sap were checked weekly. During the first 2 weeks following bud burst, remobilized N contributed ~90% of total N in growing leaves and twigs. Nitrogen uptake from the soil started concomitantly with N remobilization but contributed only slightly to bud burst. However, the fraction of total N due to N uptake increased markedly once bud burst had occurred, reaching 27% in fully expanded leaves and 18% in developed twigs. In phloem sap, the (15)N label appeared a few days after the beginning of labeling and increased until the end of bud burst, and then decreased at full leaf expansion in June. Of all the shoot compartments, leaves attracted most of the absorbed N, which accounted for 68% of new N in shoots, whereas twigs and new buds accounted for only 28 and 3%, respectively. New N allocated to leaves increased from unfolding to full expansion as total N concentration in the leaves decreased. Our results underline the crucial role played by stored N in rapid leaf growth and in the sustained growth of oak trees. Any factors that reduce N storage in autumn may therefore impair spring shoot growth.
MeSH term(s)	Analysis of Variance ; Flowers/growth & development ; Flowers/metabolism ; France ; Isotope Labeling/methods ; Linear Models ; Nitrates/metabolism ; Nitrogen/metabolism ; Nitrogen Isotopes ; Phloem/metabolism ; Plant Exudates/metabolism ; Plant Shoots/growth & development ; Plant Shoots/metabolism ; Quaternary Ammonium Compounds/metabolism ; Quercus/growth & development ; Quercus/metabolism ; Soil/chemistry ; Water/metabolism
Chemical Substances	Nitrates ; Nitrogen Isotopes ; Plant Exudates ; Quaternary Ammonium Compounds ; Soil ; Water (059QF0KO0R) ; Nitrogen (N762921K75)
Language	English
Publishing date	2011-12
Publishing country	Canada
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	743341-4
ISSN	1758-4469 ; 0829-318X
ISSN (online)	1758-4469
ISSN	0829-318X
DOI	10.1093/treephys/tpr118
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Article ; Online: Impact of hedonic evaluation on consumers' preferences for beef attributes including its enrichment with n-3 and CLA fatty acids.

Baba, Yasmina / Kallas, Zein / Costa-Font, Montserrat / Gil, José María / Realini, Carolina E

Meat science

2016 Volume 111, Page(s) 9–17

Abstract: ... origin, animal diet, fat content, color, price) including its enrichment with omega-3 (n-3) and ... conjugated linoleic acid (CLA) fatty acids. One group of consumers (n=325) received information about n-3 and CLA ... while the other group (n=322) received no information. Consumers conducted a Discrete Choice Experiment (DCE), using ...

Abstract	The impact of hedonic evaluation on consumers' preferences for beef attributes was evaluated (origin, animal diet, fat content, color, price) including its enrichment with omega-3 (n-3) and conjugated linoleic acid (CLA) fatty acids. One group of consumers (n=325) received information about n-3 and CLA, while the other group (n=322) received no information. Consumers conducted a Discrete Choice Experiment (DCE), using the recently developed Generalized Multinomial Logit model; followed by a blind hedonic evaluation of beef samples, which were identified after tasting, and finally repeated the DCE. Results showed that hedonic evaluation had a significant impact on consumers' preferences, which were similar after tasting for all consumers, with less emphasis on the fat content, color, and origin attributes and greater emphasis on animal diet. Preference for n-3 enriched beef increased, while preference for CLA enriched beef was still not significant after tasting. The information provided had a significant effect on consumers' beef preferences, but no significant impact on beef liking scores.
MeSH term(s)	Algorithms ; Animals ; Cattle ; Choice Behavior ; Consumer Health Information ; Diet/adverse effects ; Diet/veterinary ; Dietary Fats/adverse effects ; Dietary Fats/analysis ; Fatty Acids, Omega-3/administration & dosage ; Fatty Acids, Omega-3/analysis ; Fatty Acids, Omega-3/metabolism ; Food Preferences ; Food Quality ; Health Knowledge, Attitudes, Practice ; Humans ; Linoleic Acids, Conjugated/administration & dosage ; Linoleic Acids, Conjugated/analysis ; Linoleic Acids, Conjugated/metabolism ; Logistic Models ; Meat/analysis ; Meat/economics ; Models, Psychological ; Nutritive Value ; Pigments, Biological/analysis ; Pigments, Biological/biosynthesis ; Sensation ; Single-Blind Method ; Spain ; Taste
Chemical Substances	Dietary Fats ; Fatty Acids, Omega-3 ; Linoleic Acids, Conjugated ; Pigments, Biological
Language	English
Publishing date	2016-01
Publishing country	England
Document type	Comparative Study ; Journal Article ; Randomized Controlled Trial ; Research Support, Non-U.S. Gov't
ZDB-ID	753319-6
ISSN	1873-4138 ; 0309-1740
ISSN (online)	1873-4138
ISSN	0309-1740
DOI	10.1016/j.meatsci.2015.08.005
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Article ; Online: [2,2'-Imino-diethano-lato(2-)-κO,N,O'][4-(meth-oxy-carbonyl-meth-yl)phen-yl]boron.

Zein, Ahmed L / Dawe, Louise N / Georghiou, Paris E

Acta crystallographica. Section E, Structure reports online

2010 Volume 66, Issue Pt 10, Page(s) o2646

Abstract: ... boronobenzene acetate with ethano-lamine. A combination of inter-molecular N-H⋯O hydrogen bonds and C-H⋯π inter ...

Abstract	The title compound, C(13)H(18)BNO(4), was readily obtained from the reaction of methyl 4-boronobenzene acetate with ethano-lamine. A combination of inter-molecular N-H⋯O hydrogen bonds and C-H⋯π inter-actions leads to the pairwise association of mol-ecules.
Language	English
Publishing date	2010-09-30
Publishing country	United States
Document type	Journal Article
ZDB-ID	2041947-8
ISSN	1600-5368 ; 1600-5368
ISSN (online)	1600-5368
ISSN	1600-5368
DOI	10.1107/S1600536810037864
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Article: Seasonal changes of C and N non-structural compounds in the stem sapwood of adult sessile oak and beech trees

El Zein, R / Maillard, P / Bréda, N / Marchand, J / Montpied, P / Gérant, D

UNKNOWN. 2011 Aug., v. 31, no. 8

2011

Abstract: ... N) cycling of the stem rather than in N storage and remobilization to the other growing organs ...

Abstract	We assessed the pools of non-structural nitrogen compounds (NSNC) through a year, thereby addressing the question of whether mature sessile oak [Quercus petraea (Matt.) Liebl.] and beech (Fagus sylvatica L.), which differ in wood anatomy and growth patterns, exhibit contrasting seasonal dynamics of NSNC pools as previously shown for non-structural carbohydrate (NSC) pools. Seasonal fluctuations of NSNC (amino acids and soluble proteins) and NSC (starch and soluble sugars) pools were analyzed in the inner and the outer stem sapwood. In oak, NSC showed marked seasonal variation within the stem sapwood (accumulation during winter and decrease during bud burst and early wood growth), whereas in beech seasonal fluctuations in NSC were of minor amplitude. Even if the distribution and intensity of the NSNC pools differed between the two species, NSNC of the stem sapwood did not show seasonal variation. The most significant change in NSNC pools was the seasonal fluctuation of protein composition. In both species, two polypeptides of 13 kDa (PP13) and 26 kDa (PP26) accumulated during the coldest period in parallel with starch to sugar conversion and disappeared with the onset of spring growth. The absence of seasonal changes in total soluble protein concentration suggests that the polypeptides are involved in the internal nitrogen (N) cycling of the stem rather than in N storage and remobilization to the other growing organs of the tree.
Keywords	Fagus sylvatica ; Quercus petraea ; adults ; amino acids ; nitrogen ; nitrogen compounds ; polypeptides ; protein composition ; proteins ; sapwood ; seasonal variation ; seasonal wetlands ; spring ; starch ; sugars ; trees ; winter ; wood
Language	English
Dates of publication	2011-08
Size	p. 843-854.
Publishing place	Oxford University Press
Document type	Article
ZDB-ID	743341-4
ISSN	1758-4469 ; 0829-318X
ISSN (online)	1758-4469
ISSN	0829-318X
Database	NAL-Catalogue (AGRICOLA)

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Article ; Online: [2,2′-Iminodiethanolato(2−)-κ3O,N,O′][4-(methoxycarbonylmethyl)phenyl]boron

Paris E. Georghiou / Louise N. Dawe / Ahmed L. Zein

Acta Crystallographica Section E, Vol 66, Iss 10, Pp o2646-o

2010 Volume 2646

Abstract: ... acetate with ethanolamine. A combination of intermolecular N—H.O hydrogen bonds and C—H.π interactions ...

Abstract	The title compound, C13H18BNO4, was readily obtained from the reaction of methyl 4-boronobenzene acetate with ethanolamine. A combination of intermolecular N—H.O hydrogen bonds and C—H.π interactions leads to the pairwise association of molecules.
Keywords	Chemistry ; QD1-999
Language	English
Publishing date	2010-10-01T00:00:00Z
Publisher	International Union of Crystallography
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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Article: Impact of hedonic evaluation on consumers' preferences for beef attributes including its enrichment with n-3 and CLA fatty acids

Baba, Yasmina / Carolina E. Realini / José María Gil / Montserrat Costa-Font / Zein Kallas

Meat science. 2016 Jan., v. 111

2016

Abstract	The impact of hedonic evaluation on consumers' preferences for beef attributes was evaluated (origin, animal diet, fat content, color, price) including its enrichment with omega-3 (n-3) and conjugated linoleic acid (CLA) fatty acids. One group of consumers (n=325) received information about n-3 and CLA, while the other group (n=322) received no information. Consumers conducted a Discrete Choice Experiment (DCE), using the recently developed Generalized Multinomial Logit model; followed by a blind hedonic evaluation of beef samples, which were identified after tasting, and finally repeated the DCE. Results showed that hedonic evaluation had a significant impact on consumers' preferences, which were similar after tasting for all consumers, with less emphasis on the fat content, color, and origin attributes and greater emphasis on animal diet. Preference for n-3 enriched beef increased, while preference for CLA enriched beef was still not significant after tasting. The information provided had a significant effect on consumers' beef preferences, but no significant impact on beef liking scores.
Keywords	animals ; beef ; color ; conjugated linoleic acid ; consumer preferences ; diet ; lipid content ; logit analysis ; prices
Language	English
Dates of publication	2016-01
Size	p. 9-17.
Publishing place	Elsevier Ltd
Document type	Article
ZDB-ID	753319-6
ISSN	1873-4138 ; 0309-1740
ISSN (online)	1873-4138
ISSN	0309-1740
DOI	10.1016/j.meatsci.2015.08.005
Database	NAL-Catalogue (AGRICOLA)

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