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Transthyretin

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https://www.readbyqxmd.com/read/29460791/hypercholesterolemia-and-hypertriglyceridemia-as-risk-factors-of-liver-dysfunction-in-children-with-inflammation-receiving-total-parenteral-nutrition
#1
Ana Paula Brigatto Simões Golucci, André Moreno Morcillo, Taís Daiene Russo Hortencio, Antônio Fernando Ribeiro, Roberto José Negrão Nogueira
BACKGROUND AND AIMS: Hepatic dysfunction, due to parenteral nutrition, may become severe and lead to cirrhosis and hepatic failure, especially in newborns and infants. This study aimed to evaluate the association between the exclusive use of total parenteral nutrition (TPN) and changes in the hepatic profile, and to investigate the relationship between age, hypertriglyceridemia, and hypercholesterolemia, and the occurrence of laboratory liver dysfunction. METHODS: A descriptive and historical cohort study was conducted, evaluating 195 pediatric patients (age: 1 month to 19 years) who received TPN...
February 2018: Clinical Nutrition ESPEN
https://www.readbyqxmd.com/read/29449369/serum-transthyretin-predictor-of-amyloidosis-outcomes
#2
EDITORIAL
Jose Nativi-Nicolau
No abstract text is available yet for this article.
February 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29449366/use-of-serum-transthyretin-as-a-prognostic-indicator-and-predictor-of-outcome-in-cardiac-amyloid-disease-associated-with-wild-type-transthyretin
#3
Jacquelyn L S Hanson, Marios Arvanitis, Clarissa M Koch, John L Berk, Frederick L Ruberg, Tatiana Prokaeva, Lawreen H Connors
BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt), an underappreciated cause of heart failure in older adults, is challenging to diagnose and monitor in the absence of validated, disease-specific biomarkers. We examined the prognostic use and survival association of serum TTR (transthyretin) concentration in ATTRwt. METHODS AND RESULTS: Patients with biopsy-proven ATTRwt were retrospectively identified. Serum TTR, cardiac biomarkers, and echocardiographic parameters were assessed at baseline and follow-up evaluations...
February 2018: Circulation. Heart Failure
https://www.readbyqxmd.com/read/29448998/cardiac-amyloidosis-from-clinical-suspicion-to-morphological-diagnosis
#4
REVIEW
Pavla Flodrova, Patrik Flodr, Tomas Pika, Jiri Vymetal, Dusan Holub, Petr Dzubak, Marian Hajduch, Vlastimil Scudla
Amyloidosis is a heterogeneous group of diseases characterised by extracellular accumulation of amyloid in various tissues and organs of the body, leading to alteration and destruction of tissues. Heart involvement is the most important prognostic factor in patients with systemic amyloidosis and the diagnosis and typing of amyloid must be made properly. The clinical picture shows congestive heart failure with predominant right-sided heart failure symptoms in fully developed disease, various types of arrhythmias and characteristic electrocardiography and echocardiography findings...
February 12, 2018: Pathology
https://www.readbyqxmd.com/read/29442225/feeder-cell-independent-culture-of-the-pig-embryonic-stem-cell-derived-exocrine-pancreatic-cell-line-picm-31
#5
Neil C Talbot, Amy E Shannon, Caitlin E Phillips, Wesley M Garrett
The adaptation to feeder-independent growth of a pig embryonic stem cell-derived pancreatic cell line is described. The parental PICM-31 cell line, previously characterized as an exocrine pancreas cell line, was colony-cloned two times in succession resulting in the derivative cell line, PICM-31A1. PICM-31A1 cells were adapted to growth on a polymerized collagen matrix using feeder cell-conditioned medium and were designated PICM-31FF. Like the parental cells, the PICM-31FF cells were small and grew relatively slowly in closely knit colonies that eventually coalesced into a continuous monolayer...
February 13, 2018: In Vitro Cellular & Developmental Biology. Animal
https://www.readbyqxmd.com/read/29436033/rh-ii-catalyzed-intermolecular-c-h-arylation-of-aromatics-with-diazo-quinones
#6
Kai Wu, Bei Cao, Cong-Ying Zhou, Chi-Ming Che
We developed an efficient synthesis of biaryls by a dirhodium(II)-catalyzed aromatic C-H arylation with diazo quinones. The new biaryl synthesis can be performed under mild and neutral conditions and without directing group chelation assistance. The reaction tolerates various functionalities and is applicable to a broad range of aromatics. The regioselectivity of the C-H arylation was often high and predictable. The synthetic utility of the method was demonstrated by the late-stage modifications of a series of pharmaceuticals and functional materials as well as a short synthesis of a transthyretin amyloid inhibitor...
February 12, 2018: Chemistry: a European Journal
https://www.readbyqxmd.com/read/29429161/-classification-of-cardiac-amyloidosis-an-immunohistochemical-analysis
#7
L Li, X J Duan, Y Sun, Y Lu, H Y Xu, Q Z Wang, H Y Wang
Objective: To evaluate the sensitivity and specificity of immunohistochemistry (IHC) in the classification of cardiac amyloidosis on endomyocardial biopsy (EMB) and heart allograft. Methods: Twenty cardiac tissues from 19 patients at Fuwai Hospital from January, 1990 to April, 2017 with histopathologic features of amyloidosis and Congo red staining positivity were included. IHC was performed with monoclonal antibodies against AA amyloid and polyclonal antibodies against transthyretin (ATTR), λ-light chain (AL-λ), κ-light chain (AL-κ), ApoAⅠ, ApoAⅡ, ApoA Ⅳ and β(2)-microglobin...
February 8, 2018: Zhonghua Bing Li Xue za Zhi Chinese Journal of Pathology
https://www.readbyqxmd.com/read/29427707/assessment-of-endocrine-disruption-and-oxidative-potential-of-bisphenol-a-triclosan-nonylphenol-diethylhexyl-phthalate-galaxolide-and-carbamazepine-common-contaminants-of-municipal-biosolids
#8
Jo-Anne E Cavanagh, Katherine Trought, Caroline Mitchell, Grant Northcott, Louis A Tremblay
The use of biosolids as a soil conditioner and fertiliser is hindered by the limited knowledge on the risks of micro-contaminants they contain. This study investigated the binding of six organic contaminants commonly found in biosolids, to the estrogen (ER), androgen (AR), aryl hydrocarbon (AhR), and transthyretin (TTR) receptors and their redox activity. Triclosan (TCS), bisphenol-A (BPA), and technical nonylphenol (TNP) had affinity for the TTR with relative potencies of 0.3, 0.03, and 0.076 respectively...
February 7, 2018: Toxicology in Vitro: An International Journal Published in Association with BIBRA
https://www.readbyqxmd.com/read/29426546/a-chemometric-approach-for-characterization-of-serum-transthyretin-in-familial-amyloidotic-polyneuropathy-type-i-fap-i-by-electrospray-ionization-ion-mobility-mass-spectrometry
#9
Laura Pont, Victoria Sanz-Nebot, Marta Vilaseca, Joaquim Jaumot, Roma Tauler, Fernando Benavente
In this study, we describe a chemometric data analysis approach to assist in the interpretation of the complex datasets from the analysis of high-molecular mass oligomeric proteins by ion mobility mass spectrometry (IM-MS). The homotetrameric protein transthyretin (TTR) is involved in familial amyloidotic polyneuropathy type I (FAP-I). FAP-I is associated with a specific TTR mutant variant (TTR(Met30)) that can be easily detected analyzing the monomeric forms of the mutant protein. However, the mechanism of protein misfolding and aggregation onset, which could be triggered by structural changes in the native tetrameric protein, remains under investigation...
May 1, 2018: Talanta
https://www.readbyqxmd.com/read/29424556/monoclonal-gammopathy-of-undetermined-significance-in-systemic-transthyretin-amyloidosis-attr
#10
Pooja Phull, Vaishali Sanchorawala, Lawreen H Connors, Gheorghe Doros, Frederick L Ruberg, John L Berk, Shayna Sarosiek
OBJECTIVE: To identify the prevalence of monoclonal gammopathy of undetermined significance (MGUS) in patients with transthyretin (ATTR) amyloidosis. PATIENTS AND METHODS: We performed a retrospective analysis of patients with biopsy-proven ATTRwt (wild-type transthyretin amyloid protein) and genopositive ATTR V122I (valine-to-isoleucine substitution at position 122 of the TTR gene) amyloidosis evaluated at the Amyloidosis Center at Boston University and Boston Medical Center between 1 January 2003 and 31 December 2016...
February 9, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29423915/sensory-nerve-degeneration-in-a-mouse-model-mimicking-early-manifestations-of-familial-amyloid-polyneuropathy-due-to-transthyretin-ala97ser
#11
Hung-Wei Kan, Hao Chiang, Whei-Min Lin, I-Shing Yu, Shu-Wha Lin, Sung-Tsang Hsieh
AIMS: Sensory nerve degeneration and consequent abnormal sensations are the earliest and most prevalent manifestations of familial amyloid polyneuropathy (FAP) due to amyloidogenic transthyretin (TTR). FAP is a relentlessly progressive degenerative disease of the peripheral nervous system. However, there is a lack of mouse models to replicate the early neuropathic manifestations of FAP. METHODS: We established human TTR knock-in mice by replacing one allele of the mouse Ttr locus with human wild-type TTR (hTTRwt ) or human TTR with the A97S mutation (hTTRA97S )...
February 8, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29417940/inverse-changes-in-plasma-tetranectin-and-titin-levels-in-patients-with-type-2-diabetes-mellitus-a-potential-predictor-of-acute-myocardial-infarction
#12
Mohd Aizat Abdul Rahim, Zubaidah Haji Abdul Rahim, Wan Azman Wan Ahmad, Marina Mohd Bakri, Muhammad Dzafir Ismail, Onn Haji Hashim
An early intervention using biomarkers to predict acute myocardial infarction (AMI) will effectively reduce global heart attack incidence, particularly among high-risk patients with type 2 diabetes mellitus (T2DM). This study attempted to identify potential biomarkers by detecting changes in the levels of plasma proteins in T2DM patients following onset of AMI in comparison with those without AMI. Volunteer T2DM patients without AMI (control; n=10) and T2DM patients with AMI (n=10) were recruited. Plasma samples from these patients were evaluated via two-dimensional gel electrophoresis (2DE) to screen for proteins with level changes between the two groups...
February 8, 2018: Acta Pharmacologica Sinica
https://www.readbyqxmd.com/read/29414511/human-plasma-retinol-binding-protein-rbp4-is-also-a-fatty-acid-binding-protein
#13
Massimiliano Perduca, Stefania Nicolis, Barbara Mannucci, Monica Galliano, Hugo L Monaco
RBP4 (plasma retinol-binding protein) is the 21 kDa transporter of all-trans retinol that circulates in plasma as a moderately tight 1:1 molar complex of the vitamin with the protein. RBP4 is primarily synthesized in the liver but is also produced by adipose tissue and circulates bound to a larger protein, transthyretin, TTR, that serves to increase its molecular mass and thus avoid its elimination by glomerular filtration. This paper reports the high resolution three-dimensional structures of human RBP4 naturally lacking bound retinol purified from plasma, urine and amniotic fluid...
January 31, 2018: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29394116/normal-scores-of-deep-breathing-tests-beware-of-dysrhythmia-in-transthyretin-amyloidosis
#14
Urban Wiklund, Amir Kadkhodaee, Kennet Andersson, Ole B Suhr, Rolf Hörnsten
BACKGROUND: The heart rate (HR) response to paced deep breathing (DB) is a common test of cardiac autonomic function, where high heart rate variability (HRV) is considered to reflect normal autonomic function. We evaluated the DB test in patients with hereditary transthyretin amyloid (ATTRm) amyloidosis, where autonomic dysregulation and atrial arrhythmias are common. METHODS: Paced DB was performed during one minute (six breaths/min) in 165 recordings in adult ATTRm amyloidosis patients with the TTR Val30Met mutation, 42 hypertrophic cardiomyopathy (HCM) patients and 211 healthy subjects...
February 2, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29383369/on-a-chip-tryptic-digestion-of-transthyretin-a-step-toward-an-integrated-microfluidic-system-for-the-follow-up-of-familial-transthyretin-amyloidosis
#15
Jeanne Bataille, Arthur Viodé, Iago Pereiro, Josiane P Lafleur, Fanny Varenne, Stéphanie Descroix, François Becher, Jörg P Kutter, Camille Roesch, Christian Poüs, Myriam Taverna, Antoine Pallandre, Claire Smadja, Isabelle Le Potier
A microfluidic microreactor for trypsin mediated transthyretin (TTR) digestion has been developed as a step towards the elaboration of a fully integrated microdevice for the detection of a rare and disabling disease, the familial transthyretin amyloidosis (ATTR) which is related to specific TTR mutations. Therefore, an enzymatic microreactor coupled to an analytical step able to monitor the mutation of TTR on specific peptide fragments would allow an accurate monitoring of the treatment efficiency of ATTR. In this study, two types of immobilized trypsin microreactors have been investigated: a new miniaturized, microfluidic fluidized bed packed with trypsin functionalized magnetic particles (MPs), and a thiol-ene (TE) monolith-based chip...
January 31, 2018: Analyst
https://www.readbyqxmd.com/read/29369708/reduced-left-atrial-myocardial-deformation-irrespective-of-cavity-size-a-potential-cause-for-atrial-arrhythmia-in-hereditary-transthyretin-amyloidosis
#16
Michael Y Henein, Ole B Suhr, Sandra Arvidsson, Björn Pilebro, Per Westermark, Rolf Hörnsten, Per Lindqvist
BACKGROUND: Cardiac amyloidosis (CA) is a myocardial disease and commonly under-diagnosed condition. In CA patients, atrial fibrillation might occur in the absence of left atrial (LA) enlargement. OBJECTIVES: The aim of this study is to assess LA size and function, and its relationship with atrial arrhythmia in patients with hereditary transthyretin amyloidosis (ATTR). METHODS: Forty-six patients with confirmed ATTR amyloidosis on abdominal biopsy were studied...
January 25, 2018: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/29360446/a-mouse-model-of-a-novel-missense-mutation-gly83arg-in-a-chinese-kindred-manifesting-vitreous-amyloidosis-only
#17
Ling-Xia Ran, Zhi-Yong Zheng, Bing Xie, Xiao-Mei Nie, Xing-Wang Chen, Gang Su, Shan-Jun Cai
The purpose of this study is to establish a mouse model of transthyretin (TTR) Gly83Arg gene mutation by the technique of gene targeting for research on hereditary vitreous amyloidosis (HVA) and to confirm whether this point mutation is a genetic feature of HVA. A vector (pBR322-MK-TTR) was constructed to target ES cells. The successfully transfected ES cells were used for blastocyst injection, thus generating F0. F0 and Flp mice were mated to generate F1 (TTR+/-, Flp +/-) mice that lacked the neo gene but carried the Flp gene...
January 19, 2018: Experimental Eye Research
https://www.readbyqxmd.com/read/29351628/-a-new-staging-system-for-cardiac-transthyretin-amyloidosis-is-it-already-on-the-verge-of-obsolescence
#18
Avinainder Singh, Rodney H Falk
No abstract text is available yet for this article.
January 16, 2018: European Heart Journal
https://www.readbyqxmd.com/read/29346382/6-ohda-induced-dopaminergic-neurodegeneration-in-caenorhabditis-elegans-is-promoted-by-the-engulfment-pathway-and-inhibited-by-the-transthyretin-related-protein-ttr-33
#19
Sarah-Lena Offenburger, Xue Yan Ho, Theresa Tachie-Menson, Sean Coakley, Massimo A Hilliard, Anton Gartner
Oxidative stress is linked to many pathological conditions including the loss of dopaminergic neurons in Parkinson's disease. The vast majority of disease cases appear to be caused by a combination of genetic mutations and environmental factors. We screened for genes protecting Caenorhabditis elegans dopaminergic neurons from oxidative stress induced by the neurotoxin 6-hydroxydopamine (6-OHDA) and identified the transthyretin-related gene ttr-33. The only described C. elegans transthyretin-related protein to date, TTR-52, has been shown to mediate corpse engulfment as well as axon repair...
January 2018: PLoS Genetics
https://www.readbyqxmd.com/read/29344917/non-cardiac-uptake-of-technetium-99m-pyrophosphate-in-transthyretin-cardiac-amyloidosis
#20
Brett W Sperry, Matthew H Gonzalez, Richard Brunken, Manuel D Cerqueira, Mazen Hanna, Wael A Jaber
BACKGROUND: Technetium-based bone scintigraphy is rapidly becoming the most common non-invasive imaging tool in the diagnosis of Transthyretin cardiac amyloidosis (ATTR). Skeletal muscle uptake has been described with technetium-99m-3,3-diphosphono-1,2-propanodicarboxylic acid (TcDPD), and may account for masking of bony uptake. We sought to investigate skeletal muscle uptake of technetium-99m-pyrophosphate (TcPYP) in patients with ATTR. METHODS AND RESULTS: This was a retrospective analysis of 57 patients diagnosed with ATTR who underwent TcPYP scintigraphy...
January 17, 2018: Journal of Nuclear Cardiology: Official Publication of the American Society of Nuclear Cardiology
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