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https://read.qxmd.com/read/30770754/glycomacropeptide-long-term-use-and-impact-on-blood-phenylalanine-growth-and-nutritional-status-in-children-with-pku
#1
REVIEW
A Daly, S Evans, S Chahal, S Santra, A Pinto, R Jackson, C Gingell, J Rocha, F J Van Spronsen, A MacDonald
In phenylketonuria, casein glycomacropeptide (CGMP) requires modification with the addition of some essential and semi essential amino acids to ensure suitability as a protein substitute. The optimal amount and ratio of additional amino acids is undefined. AIM: A longitudinal, parallel, controlled study over 12 months evaluating a CGMP (CGMP-AA2) formulation compared with phenylalanine-free L-amino acid supplements (L-AA) on blood Phe, Tyr, Phe:Tyr ratio, biochemical nutritional status and growth in children with PKU...
February 15, 2019: Orphanet Journal of Rare Diseases
https://read.qxmd.com/read/30766595/the-immunomodulatory-peptide-bursopentin-bp5-enhances-proliferation-and-induces-sigm-expression-in-dt40-cells
#2
Xiang-Bo Ji, Jun Luo, Xiu-Li Feng, Qiu-Liang Xu, Teng Man, Dong Zhao, Xin-Feng Li, Gai-Ping Zhang, Pu-Yan Chen
Background: In the recent past, many studies have been focused on extracts of BF and multiple biologically active factors and their effects on humoral immune system in chickens and birds. However, the mechanism of those immunomodulatory peptides on the B lineage cells proliferation and antibody production in chicken is fairly unknown. DT40 cell line, an avian leucosis virus-induced chicken pre-B cell line, expresses immunoglobulin M (IgM) isotype B cell reporter in the plasma membrane...
December 2018: African Health Sciences
https://read.qxmd.com/read/30763665/characterization-of-a-mutation-in-the-zona-pellucida-module-of-endoglin-that-causes-hereditary-hemorrhagic-telangiectasia
#3
Lidia Ruiz-Llorente, Elisa Chiapparino, Sara Plumitallo, Cesare Danesino, Pinar Bayrak-Toydemir, Fabio Pagella, Guido Manfredi, Carmelo Bernabeu, Luca Jovine, Carla Olivieri
Hereditary hemorrhagic telangiectasia (HHT) is a vascular rare disease characterized by nose and gastrointestinal bleeding, skin and mucosa telangiectasias, and arteriovenous malformations in internal organs. HHT shows an autosomal dominant inheritance and a worldwide prevalence of approximately 1:5000 individuals. In >80% of patients, HHT is caused by mutations in either ENG (HHT1) or ACVRL1 (HHT2) genes, which code for the membrane proteins Endoglin and Activin A Receptor Type II-Like Kinase 1 (ALK1), respectively, both belonging to the TGF-β/BMP signaling pathway...
February 11, 2019: Gene
https://read.qxmd.com/read/30716167/mocha-tyrosinase-variant-a-new-flavour-of-cat-coat-coloration
#4
Y Yu, R A Grahn, L A Lyons
A novel coloration named 'mocha' has been identified in the Burmese cat breed from Thailand. Tyrosinase (TYR) mutations are known to be associated with coat coloration in cats, such as the sable Burmese, the points of the Siamese and albino cats. Additionally, sable Burmese that produced mocha-colored cats had unexpected genotypes for TYR. Therefore, TYR was considered a candidate gene for mocha in cats. Sanger sequencing for genomic DNA revealed NC_018732.3:chromosome D1:45 898 609_45 898 771dup in exon 2 and intron 2 of TYR...
February 4, 2019: Animal Genetics
https://read.qxmd.com/read/30695994/-leathesia-difformis-extract-inhibits-%C3%AE-msh-induced-melanogenesis-in-b16f10-cells-via-down-regulation-of-creb-signaling-pathway
#5
Ga-Young Seo, Yuna Ha, Ah-Hyun Park, Oh Wook Kwon, Youn-Jung Kim
Leathesia difformis (L.) Areschoug ( L. difformis ) is a species of littoral brown algae of the class Phaeophyceae. Only a few studies on the apoptotic, antiviral, and antioxidant properties of L. difformis have been reported, and its inhibitory effect on melanin synthesis has not been studied. The aim of this study was to investigate the anti-melanogenic effect of L. difformis extract on α-melanocyte-stimulating hormone (α-MSH)-induced B16F10 melanocytes and its mechanism of action. L. difformis was extracted using 80% ethanol (LDE) and then fractioned between ethyl acetate (LDE-EA) and water (LDE-A)...
January 28, 2019: International Journal of Molecular Sciences
https://read.qxmd.com/read/30689908/extra-dietary-protein-bound-or-free-amino-acids-differently-affect-the-serum-concentrations-of-free-amino-acids-in-heat-stressed-pigs
#6
A Morales, M Chávez, N Vásquez, L Camacho, E Avelar, N Arce, J K Htoo, M Cervantes
Pigs exposed to heat stress (HS) reduce feed intake and consequently the consumption of amino acids (AA). Adding extra protein-bound or free AA to the diet may correct the reduced AA intake of HS pigs. However, extra protein-bound AA may further increase the body heat load whereas extra free AA does not affect the heat load of HS pigs. Two experiments were conducted. In Exp. 1, the performance depression because of HS, compared with thermal neutrality, was determined with 30 pigs (31.1 ± 1.2 kg BW) fed diets with AA only as protein or as a mix of protein and free AA...
January 21, 2019: Journal of Animal Science
https://read.qxmd.com/read/30680790/phenotypic-and-genotypic-analysis-of-amelanotic-and-hypomelanotic-melanoma-patients
#7
J E Rayner, E K McMeniman, D L Duffy, B De'Ambrosis, B M Smithers, K Jagirdar, K J Lee, H P Soyer, R A Sturm
BACKGROUND: Amelanotic/hypomelanotic melanoma is associated with poorer outcomes due to a more advanced disease stage at diagnosis. OBJECTIVE: To determine phenotypic risks and genotypic associations with amelanotic/hypomelanotic melanoma to develop a clinical and genetic profile that could assist in identifying high-risk individuals. METHODS: The Brisbane Naevus Morphology Study conducted from 2009-2016 has recruited a core of 1254 participants...
January 24, 2019: Journal of the European Academy of Dermatology and Venereology: JEADV
https://read.qxmd.com/read/30679655/a-pathogenic-haplotype-common-in-europeans-causes-autosomal-recessive-albinism-and-uncovers-missing-heritability-in-oca1
#8
Karen Grønskov, Cathrine Jespersgaard, Gitte Hoffmann Bruun, Pernille Harris, Karen Brøndum-Nielsen, Brage S Andresen, Thomas Rosenberg
Oculocutaneous albinism (OCA) is a genetically heterogeneous disorder. Six genes are associated with autosomal recessive OCA (TYR, OCA2, TYRP1, SLC45A2, SLC24A5 and LRMDA), and one gene, GPR143, is associated with X-linked ocular albinism (OA). Molecular genetic analysis provides a genetic diagnosis in approximately 60% of individuals with clinical OA/OCA. A considerably number of the remaining 40% are heterozygous for a causative sequence variation in TYR. To identify missing causative sequence variants in these, we used a NGS based approach, genotyping and segregation analysis...
January 24, 2019: Scientific Reports
https://read.qxmd.com/read/30675637/engineering-clps-for-selective-and-enhanced-n-terminal-amino-acid-binding
#9
Jennifer Tullman, Nicholas Callahan, Benjamin Ellington, Zvi Kelman, John P Marino
One of the central challenges in the development of single-molecule protein sequencing technologies is achieving high-fidelity sequential recognition and detection of specific amino acids that comprise the peptide sequence. An approach towards achieving this goal is to leverage naturally occurring proteins that function through recognition of amino (N)-terminal amino acids (NAAs). One such protein, the N-end rule pathway adaptor protein ClpS, natively recognizes NAAs on a peptide chain. The native ClpS protein has a high specificity albeit modest affinity for the amino acid Phe at the N-terminus but also recognizes the residues Trp, Tyr, and Leu at the N-terminal position...
January 24, 2019: Applied Microbiology and Biotechnology
https://read.qxmd.com/read/30666904/domain-specific-stabilization-of-structural-and-dynamic-responses-of-human-serum-albumin-by-sucrose
#10
Vaisakh Mohan, Bhaswati Sengupta, Nilimesh Das, Indrani Banerjee, Pratik Sen
In the present work, we have investigated the denaturing and renaturing effects of urea and sucrose respectively, on the overall structure and on two different domains (domain-I and domain-III) of multi-domain human serum albumin (HSA). From circular dichroism measurements it could be seen that, the α-helicity of the overall structure, which is 65 % in its native state, decreases to 11 % in presence of 9 M urea and further increases to 25 % with the addition of 1 M sucrose. In order to study the domain-specific responses towards these external agents, HSA was covalently tagged with fluorescent probes N-(7-dimethylamino-4-methylcoumarin-3-yl) iodoacetamide (DACIA) and p-nitrophenyl coumarin ester (NPCE) at Cys-34 of domain-I and Tyr-411 of domain-III, respectively...
January 21, 2019: Protein and Peptide Letters
https://read.qxmd.com/read/30649334/maternal-nutrition-and-stage-of-early-pregnancy-in-beef-heifers-impacts-on-hexose-and-aa-concentrations-in-maternal-and-fetal-fluids
#11
Matthew S Crouse, Nathaniel P Greseth, Kyle J McLean, Mellissa R Crosswhite, Nicolas Negrin Pereira, Alison K Ward, Lawrence P Reynolds, Carl R Dahlen, Bryan W Neville, Pawel P Borowicz, Joel S Caton
We examined the hypothesis that maternal nutrition and day of gestation would affect the concentrations of amino acids (AA) and hexoses in bovine utero-placental fluids and maternal serum from d 16 to 50 of gestation. Forty-nine cross-bred Angus heifers were bred via artificial insemination and fed a control diet (CON = 100% of requirements for growth) or a restricted diet (RES = 60% of CON) and ovariohysterectomized on d 16, 34, or 50 of gestation; non-pregnant (NP) controls were not bred and ovariohysterectomized on d 16 of the synchronized estrous cycle...
January 10, 2019: Journal of Animal Science
https://read.qxmd.com/read/30638233/non-covalent-complexes-of-the-peptide-fragment-gly-asn-asn-gln-gln-asn-tyr-in-the-gas-phase-photodissociative-cross-linking-born-oppenheimer-molecular-dynamics-and-ab-initio-computational-binding-study
#12
Shu R Huang, Yang Liu, František Tureček
Non-covalent complexes of the short amyloid peptide motif Gly-Asn-Asn-Gln-Gln-Asn-Tyr (GNNQQNY) with peptide counterparts that were tagged with a diazirine ring at the N-termini (*GNNQQNY) were generated as singly charged ions in the gas phase. Specific laser photodissociation (UVPD) of the diazirine tag in the gas-phase complexes at 355 nm generated transient carbene intermediates that underwent covalent cross-linking with the target GNNQQNY peptide. The crosslinking yields ranged between 0.8 and 4.5%, depending on the combinations of peptide C-terminal amides and carboxylates...
January 14, 2019: Physical Chemistry Chemical Physics: PCCP
https://read.qxmd.com/read/30623207/monitoring-activities-of-receptor-tyrosine-kinases-using-a-universal-adapter-in-genetically-encoded-split-tev-assays
#13
Jan P Wintgens, Sven P Wichert, Luksa Popovic, Moritz J Rossner, Michael C Wehr
Receptor tyrosine kinases (RTKs) play key roles in various aspects of cell biology, including cell-to-cell communication, proliferation and differentiation, survival, and tissue homeostasis, and have been implicated in various diseases including cancer and neurodevelopmental disorders. Ligand-activated RTKs recruit adapter proteins through a phosphotyrosine (p-Tyr) motif that is present on the RTK and a p-Tyr-binding domain, like the Src homology 2 (SH2) domain found in adapter proteins. Notably, numerous combinations of RTK/adapter combinations exist, making it challenging to compare receptor activities in standardised assays...
January 8, 2019: Cellular and Molecular Life Sciences: CMLS
https://read.qxmd.com/read/30608101/bacterial-biofilm-destruction-by-size-surface-charge-adaptive-micelles
#14
Maohua Chen, Jiaojun Wei, Songzhi Xie, Xinyan Tao, Zhanlin Zhang, Pan Ran, Xiaohong Li
Biofilms formed by pathogenic bacteria are one of the most important reasons for multidrug resistance. One of the major limitations in the biofilm treatment is the existence of intensive matrices, which greatly block the diffusion of antimicrobial agents. In the current study, we designed poly(aspartamide)-derived micelles self-assembled from cationic copolymers with azithromycin-conjugated and pH-sensitive copolymers, followed by loading cis-aconityl-d-tyrosine (CA-Tyr) via electrostatic interactions. In response to the acidic microenvironment of the biofilm matrix, the hydrophilic transition of the pH-sensitive copolymers and the removal of CA-Tyr led to a sharp decrease in micelle size from 107 nm to 54 nm and a rapid shift in their zeta potential from -11...
January 17, 2019: Nanoscale
https://read.qxmd.com/read/30590496/effect-of-fluoroquinolone-resistance-mutation-thr-82%C3%A2-ile-on-clostridioides-difficile-fitness
#15
J J Vernon, M H Wilcox, J Freeman
Objectives: Fluoroquinolone resistance is common among epidemic Clostridioides difficile PCR ribotype (RT) 027 and may have contributed to outbreaks of C. difficile infection (CDI). We investigated the impact of fluoroquinolone mutations on the bacterial fitness (BF) of C. difficile RT027 isolates. Methods: The BF of seven RT027 mutants with reduced susceptibility to moxifloxacin (moxifloxacin MIC 4-32 mg/L) was compared with their susceptible (moxifloxacin MIC 1-2 mg/L) progenitor strains in competitive batch culture (CBC), cell cytotoxicity and maximal growth rate assays...
December 24, 2018: Journal of Antimicrobial Chemotherapy
https://read.qxmd.com/read/30582909/the-antihyperalgesic-effect-of-docosahexaenoic-acid-in-streptozotocin-induced-neuropathic-pain-in-the-rat-involves-the-opioidergic-system
#16
Arizai Yolia Landa-Juárez, Francisca Pérez-Severiano, Gilberto Castañeda-Hernández, Mario I Ortiz, Aracely Evangelina Chávez-Piña
Docosahexaenoic acid (DHA) is a polyunsaturated fatty acid that has shown an antinociceptive effect in multiple pain models, such as inflammatory and neuropathic pain by chronic constriction injury in rats; however, its mechanism of action is still not well-understood. Reports suggest that DHA activates opioid signaling, but there is no information on this from a model of neuropathic pain. As a result, the aims of this study were (1) to determine the antihyperalgesic and antiallodynic effect of peripheral DHA administration, and (2) to evaluate the participation of the opioid receptors in the antihyperalgesic effect of DHA on streptozotocin-induced neuropathic pain in the rat...
December 21, 2018: European Journal of Pharmacology
https://read.qxmd.com/read/30572274/interaction-of-ouabain-and-progesterone-on-induction-of-bull-sperm-capacitation
#17
Mrudhula Sajeevadathan, Murray J Pettitt, Mary Buhr
The endogenous steroid hormone ouabain induces capacitation of bull sperm acting through its receptor Na+ /K+ -ATPase on the sperm plasma membrane. Progesterone (P4) is believed to act through the sperm membrane P4 receptor (mPR) to induce non-genomic signalling leading to capacitation and/or acrosome reaction (AR) in the sperm of some species, but the exact nature of this receptor molecule on bull sperm is not known. In amphibian oocytes, P4 acts through the low-affinity ouabain binding site on Na+ /K+ -ATPase to induce signalling highly reminiscent of ouabain's signalling that initiates capacitation...
December 3, 2018: Theriogenology
https://read.qxmd.com/read/30566595/effect-of-inclusion-level-of-corn-germ-meal-on-the-digestible-and-metabolizable-energy-and-evaluation-of-ileal-amino-acid-digestibility-of-corn-germ-meal-fed-to-growing-pigs
#18
Z Y Zhang, Z Y Liu, S Zhang, C H Lai, D L Ma, C F Huang
Two experiments were conducted to investigate the effect of inclusion level of corn germ meal (CGM) on the DE and ME values of CGM and to evaluate the ileal AA digestibility of CGM fed to growing pigs. In Exp. 1, 42 barrows (63.8 ± 2.1 kg BW) were allotted to 7 diets in a completely randomized design with 6 replicates per diet. Diets included a corn-soybean meal (SBM) diet and 6 additional diets containing 4.85, 9.70, 19.40, 29.10, 38.80, or 48.50% CGM. Pigs were fed twice daily, at 0730 and 1630 h, at a level of 4% of BW, and feces and urine were collected for 5 d...
December 19, 2018: Journal of Animal Science
https://read.qxmd.com/read/30564763/increased-dietary-intake-of-tyrosine-upregulates-melanin-deposition-in-the-hair-of-adult-black-coated-dogs
#19
Adrian Watson, Jamie Wayman, Russell Kelley, Alexandre Feugier, Vincent Biourge
The principle determinant of melanin derived hair colour and patterning in mammals is genetic, but environmental factors are now thought to play a role. It has been shown that the concentration of melanins in cat hair is influenced by the amino acid composition of their diets. Also, puppies were found to require tyrosine (Tyr) intake significantly greater than that recommended for normal growth and development in order to optimize melanin expression in their coats. Much of the work to date has been conducted in growing animals...
December 2018: Animal nutrition
https://read.qxmd.com/read/30558096/a-case-report-co-occurrence-of-wilson-disease-and-oculocutaneous-albinism-in-a-chinese-patient
#20
Rao Rao, Shan Shu, Yong Zhu Han, Yu-Jen Chiu, Yong Sheng Han
RATIONALE: Both Wilson disease (WD) and Oculocutaneous Albinism (OCA) are rare autosomal recessive disorders that are caused by mutations on chromosome 13 and chromosome 11, respectively. Here, we report on a patient with coexisting WD and OCA, initially presenting episodes of tremors. PATIENT CONCERNS: WD is a disorder of copper metabolism. The main sites of copper accumulation are the liver and the brain, resulting in hepatic symptoms. OCA is a disorder of melanin biosynthesis, characterized by a generalized reduction in pigmentation of the eyes (oculo-), skin (-cutaneous), and hair...
December 2018: Medicine (Baltimore)
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