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Amyloid: the International Journal of Experimental and Clinical Investigation

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https://www.readbyqxmd.com/read/28719238/mining-databases-for-protein-aggregation-a-review
#1
Paraskevi L Tsiolaki, Katerina C Nastou, Stavros J Hamodrakas, Vassiliki A Iconomidou
Protein aggregation is an active area of research in recent decades, since it is the most common and troubling indication of protein instability. Understanding the mechanisms governing protein aggregation and amyloidogenesis is a key component to the aetiology and pathogenesis of many devastating disorders, including Alzheimer's disease or type 2 diabetes. Protein aggregation data are currently found "scattered" in an increasing number of repositories, since advances in computational biology greatly influence this field of research...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28719236/applying-an-artificial-neural-network-model-for-developing-a-severity-score-for-patients-with-hereditary-amyloid-polyneuropathy
#2
Shenia Novis, Felipe Machado, Victor B Costa, Debora Foguel, Marcia W Cruz, José Manoel de Seixas
Hereditary (familial) amyloid polyneuropathy (FAP) is a systemic disease that includes a sensorimotor polyneuropathy related to transthyretin (TTR) mutations. So far, a scale designed to classify the severity of this disease has not yet been validated. This work proposes the implementation of an artificial neural network (ANN) in order to develop a severity scale for monitoring the disease progression in FAP patients. In order to achieve this goal, relevant symptoms and laboratory findings were collected from 98 Brazilian patients included in THAOS - the Transthyretin Amyloidosis Outcomes Survey...
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28719235/artificial-neural-network-modelling-of-attr-amyloidosis-is-now-the-time
#3
John L Berk
No abstract text is available yet for this article.
July 18, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28699800/towards-the-improvement-in-stability-of-an-anti-a%C3%AE-single-chain-variable-fragment-scfv-h3d6-as-a-way-to-enhance-its-therapeutic-potential
#4
Laia Montoliu-Gaya, Javier Murciano-Calles, Jose C Martinez, Sandra Villegas
ScFv-h3D6 is a single-chain variable fragment derived from the monoclonal antibody bapineuzumab that prevents Aβ-induced cytotoxicity by capturing Aβ oligomers. The benefits of scFv-h3D6 treatment in Alzheimer's disease are known at the behavioural, cellular and molecular levels in the 3xTg-AD mouse model. Antibody-based therapeutics are only stable in a limited temperature range, so their benefit in vivo depends on their capability for maintaining the proper fold. Here, we have stabilized the scFv-h3D6 folding by introducing the mutation VH-K64R and combining it with the previously described elongation of the VL domain (C3)...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28699793/predictors-of-early-treatment-failure-following-initial-therapy-for-systemic-immunoglobulin-light-chain-amyloidosis
#5
Nidhi Tandon, Surbhi Sidana, S Vincent Rajkumar, Angela Dispenzieri, Morie A Gertz, Martha Q Lacy, Robert A Kyle, Francis K Buadi, David Dingli, Suzanne R Hayman, Amie L Fonder, Miriam A Hobbs, Wilson I Gonsalves, Prashant Kapoor, Yi Lisa Hwa, Nelson Leung, Ronald S Go, John A Lust, Stephen J Russell, Steven R Zeldenrust, Shaji K Kumar
We analysed factors predicting early treatment failure (ETF), after first-line therapy for light-chain amyloidosis (AL). AL amyloidosis patients seen at Mayo Clinic within 90 days of diagnosis, from 2006 to 2015, excluding those who died within 3 months of initial therapy, were analysed retrospectively. ETF was defined as progression requiring treatment change or death within 12 (ETF12) or 24 (ETF24) months of first-line treatment. Non-ETF included those with a follow-up of more than 12 or 24 months who had progression beyond 12 or 24 months...
July 12, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28686525/kidney-biopsy-in-aa-amyloidosis-impact-of-histopathology-on-prognosis
#6
Zeynep Kendi Celebi, Saba Kiremitci, Bengi Ozturk, Serkan Akturk, Siyar Erdogmus, Neval Duman, Kenan Ates, Sehsuvar Erturk, Gokhan Nergizoglu, Sim Kutlay, Sule Sengul, Arzu Ensari, Kenan Keven
In AA amyloidosis, while kidney biopsy is widely considered for diagnosis by clinicians, there is no evidence that the detailed investigation of renal histopathology can be utilized for the prognosis and clinical outcomes. In this study, we aimed to obtain whether histopathologic findings in kidney biopsy of AA amyloidosis might have prognostic and clinical value. This is a retrospective cohort study that included 38 patients who were diagnosed with AA amyloidosis by kidney biopsy between 2005 and 2013.The kidney biopsy specimens of patients were evaluated and graded for several characteristics of histopathological lesions and their relationship with renal outcomes...
July 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28686088/changing-epidemiology-of-aa-amyloidosis-clinical-observations-over-25-years-at-a-single-national-referral-centre
#7
Thirusha Lane, Jennifer H Pinney, Janet A Gilbertson, David F Hutt, Dorota M Rowczenio, Shameem Mahmood, Sajitha Sachchithanantham, Marianna Fontana, Taryn Youngstein, Candida C Quarta, Ashutosh D Wechalekar, Julian D Gillmore, Philip N Hawkins, Helen J Lachmann
OBJECTIVE: Systemic AA amyloidosis is a serious complication of chronic inflammation; however, there are relatively few published data on its incidence. We investigated the changing epidemiology of AA amyloidosis over a 25-year period at a single national referral centre. METHODS: We conducted a retrospective study of all patients diagnosed with AA amyloidosis who had attended the centre between 1990 and 2014 inclusive. Six hundred and twenty-five patients were studied in three cohorts: C1: 1990-1997; C2: 1998-2006; C3: 2007-2014...
July 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28632454/localized-amyloidogenic-immunoglobulin-light-chain-derived-amyloidosis-in-a-young-boy-and-an-adolescent-girl
#8
Shu-Ichi Ikeda
No abstract text is available yet for this article.
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28632419/in-vitro-co-expression-of-human-amyloidogenic-immunoglobulin-light-and-heavy-chain-proteins-a-relevant-cell-based-model-of-al-amyloidosis
#9
Elena S Klimtchuk, Tatiana B Prokaeva, Brian H Spencer, Olga Gursky, Lawreen H Connors
Immunoglobulin (Ig) light chain (LC) amyloidosis (AL) is characterized by the overproduction and tissue deposition of monoclonal LC in various organs and tissues. The plasma circulating monoclonal LC is believed to be the precursor of the deposited protein and in vitro studies aimed at understanding AL pathobiology have mainly focused on LC and its variable domain. While 33% of patients have free circulating monoclonal LC, ∼40% feature LC complexed to heavy chain (HC) forming a monoclonal intact Ig; the significance of free vs...
June 20, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28613962/significant-association-between-renal-function-and-area-of-amyloid-deposition-in-kidney-biopsy-specimens-in-both-aa-amyloidosis-associated-with-rheumatoid-arthritis-and-al-amyloidosis
#10
Takeshi Kuroda, Naohito Tanabe, Eriko Hasegawa, Ayako Wakamatsu, Yukiko Nozawa, Hiroe Sato, Takeshi Nakatsue, Yoko Wada, Yumi Ito, Naofumi Imai, Mitsuhiro Ueno, Masaaki Nakano, Ichiei Narita
The kidney is a major target organ for systemic amyloidosis, which results in proteinuria and an elevated serum creatinine level. The clinical manifestations and precursor proteins of amyloid A (AA) and light-chain (AL) amyloidosis are different, and the renal damage due to amyloid deposition also seems to differ. The purpose of this study was to clarify haw the difference in clinical features between AA and AL amyloidosis are explained by the difference in the amount and distribution of amyloid deposition in the renal tissues...
June 14, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28598686/how-your-ears-can-tell-what-is-hidden-in-your-heart-wild-type-transthyretin-amyloidosis-as-potential-cause-of-sensorineural-hearing-loss-inelderly-amylodeafness-pilot-study
#11
Emilie Béquignon, Aziz Guellich, Sophie Barthier, Marc Raynal, Virginie Prulière-Escabasse, Florence Canouï-Poitrine, André Coste, Thibaud Damy
BACKGROUND: Wild-type transthyretin amyloidosis (ATTRwt) is an age-related life-threatening condition. Prognosis is mainly dependent on cardiac involvement. Other organs and tissues may be affected. Their early recognition may increase awareness of physicians and positively affects the prognosis. Presbycusis is another age-related disorder. Whether this disease is associated to ATTRwt amyloidosis is unknown. METHODS: Sixteen consecutive patients with confirmed diagnosis of ATTRwt amyloidosis at the Mondor Amyloidosis Network, France, underwent otoscopy and audiological tests including pure tone audiometry, speech reception threshold and speech discrimination score...
June 9, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28590781/cognitive-impairment-in-liver-transplanted-patients-with-transthyretin-related-hereditary-amyloid-polyneuropathy
#12
Vanda Freitas Castro, Pedro Nascimento Alves, Ana Catarina Franco, Isabel Pavão Martins, Isabel Conceição
INTRODUCTION AND AIM: Hereditary transthyretin-related amyloidosis (ATTR-FAP) is characterized by a progressive neuropathy, cardiomyopathy, nephropathy and ocular disease. More than 90% of amyloidogenic transthyretin is produced by the liver; however, this protein is also synthesized in the choroid plexus. Although some patients have transitory neurologic events, the impact on cognition is still unknown. The aim was to study the cognitive performance of ATTR-FAP V30M patients with long disease course...
June 7, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28562083/cardiac-light-chain-deposition-disease-relapsing-in-the-transplanted-heart
#13
Alberto Aimo, Giuseppe Vergaro, Angela Pucci, Sonia Bernazzali, Massimo Maccherini, Gabriele Buda, Claudio Passino, Giampaolo Merlini, Michele Emdin
No abstract text is available yet for this article.
May 31, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28553897/diagnostic-score-for-the-detection-of-cardiac-amyloidosis-in-patients-with-left-ventricular-hypertrophy-and-impact-on-prognosis
#14
Eve Cariou, Youssef Bennani Smires, Gérard Victor, Guillaume Robin, David Ribes, Pierre Pascal, Antoine Petermann, Pauline Fournier, Stanislas Faguer, Jérôme Roncalli, Hervé Rousseau, Dominique Chauveau, Didier Carrié, Isabelle Berry, Michel Galinier, Olivier Lairez
BACKGROUND: Among diagnosis associated with left ventricular hypertrophy (LVH), cardiac amyloidosis (CA) is a progressive disease with poor prognosis. Early noninvasive identification is of growing clinical importance. The objective of our study was to integrate clinical, biologic, electrocardiographic and echocardiographic parameters to build a diagnostic score in patients with LVH. METHODS AND RESULTS: One hundred and fourteen patients with LVH underwent a cardiac magnetic resonance (CMR) and a (99m)Tc-hydroxymethylene-diphosphonate scintigraphy ((99m)Tc-HMDP) allowing to discriminate three groups of diagnoses: CA (n = 50 including 31, 18 and 1 ATTR, AL and AA amyloidosis), hypertrophic cardiomyopathy (n = 19) and unspecific cardiomyopathy (n = 45)...
May 29, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28532173/cervicomedullary-compression-as-the-main-manifestation-of-wild-type-transthyretin-amyloidosis
#15
Kourosh Rezania, Peter Pytel, W Edward Highsmith, Patrik Gabikian
No abstract text is available yet for this article.
May 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28494620/genetic-testing-improves-identification-of-transthyretin-amyloid-attr-subtype-in-cardiac-amyloidosis
#16
Emily E Brown, Yi Zhen Joan Lee, Marc K Halushka, Charles Steenbergen, Nicole M Johnson, Johana Almansa, Ryan J Tedford, Oscar Cingolani, Stuart D Russell, Kavita Sharma, Daniel P Judge
Amyloidosis is a group of conditions characterized by the accumulation of amyloid deposits in various tissues. Among these disorders, ATTR amyloidosis occurs either with or without a TTR pathogenic variant. Treatment for amyloidosis depends on the subtype, which is often identified through a tissue biopsy followed by liquid chromatography tandem mass spectrometry (LC-MS/MS). Genetic testing may be done to confirm these results for patients with ATTR amyloidosis; however, the necessity of genetic testing after LC-MS/MS has not been evaluated...
May 11, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28488941/a-v-block-as-presentation-of-cardiac-amyloid-prominent-infiltration-of-conduction-tissue-revealed-by-endomyocardial-biopsy
#17
Andrea Frustaci, Claudio Letizia, Francesco Adamo, Claudia Grande, Romina Verardo, Cristina Chimenti
No abstract text is available yet for this article.
May 10, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28485639/cathepsin-k-as-a-novel-amyloid-fibril-protein-in-humans
#18
Reinhold P Linke, Louise C Serpell, Friedrich Lottspeich, Mitsuyasu Toyoda
No abstract text is available yet for this article.
May 9, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28475415/transthyretin-amyloidosis-a-phenocopy-of-hypertrophic-cardiomyopathy
#19
Alexa M C Vermeer, Anneloes Janssen, Peter C Boorsma, Marcel M A M Mannens, Arthur A M Wilde, Imke Christiaans
OBJECTIVES: Hypertrophic cardiomyopathy (HCM) is an inherited cardiac disorder that affects over one in 500 persons worldwide. The autosomal dominant transmission of HCM implies that many relatives are at risk for HCM associated morbidity and mortality, therefore genetic testing and counselling is of great importance. However, in only 50-60% of the patients a mutation is found, which hampers predictive genetic testing in relatives. In HCM patients in whom the causal mutation has not been identified (yet), phenocopies of HCM - i...
May 5, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
https://www.readbyqxmd.com/read/28434267/amyloid-in-bone-marrow-smears-in-systemic-light-chain-amyloidosis
#20
Christoph Kimmich, Stefan Schönland, Sandra Kräker, Mindaugas Andrulis, Anthony D Ho, Gudrun Mayer, Tobias Dittrich, Michael Hundemer, Ute Hegenbart
We performed a prospective sensitivity analysis to detect amyloid in bone marrow (BM) smears stained with Congo red (CR) and according to Pappenheim of patients with systemic light-chain (AL) amyloidosis. Results were directly compared to routine BM histology and fat aspiration. We analysed 198 BM smears from patients with the diagnosis or suspicion of systemic AL amyloidosis. Ultimately, the diagnosis could be established for 168 patients. Amyloid was detected on BM smears with CR in 33% (56/168). All patients suspicious for amyloid on Pappenheim staining (n = 39) showed substantial amyloid infiltration on CR...
April 23, 2017: Amyloid: the International Journal of Experimental and Clinical Investigation
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