A5032704557- Amyloidosis: Diagnosis, Treatment, Outcomes
- Parathyroid Disorders and Treatments
- Mass Spectrometry Techniques and Applications
- X-ray Diffraction in Crystallography
- Crystallization and Solubility Studies
- Chemical Reaction Mechanisms
- Cellular transport and secretion
- Medical Imaging and Pathology Studies
- Advanced Chemical Physics Studies
- Protein Kinase Regulation and GTPase Signaling
- Advanced Polymer Synthesis and Characterization
- Analytical Chemistry and Chromatography
- Free Radicals and Antioxidants
- Advanced Materials and Mechanics
- Spectroscopy and Quantum Chemical Studies
- Adsorption, diffusion, and thermodynamic properties of materials
- Molecular spectroscopy and chirality
- Various Chemistry Research Topics
- Autoimmune Bullous Skin Diseases
- Coagulation, Bradykinin, Polyphosphates, and Angioedema
- Dermatological and Skeletal Disorders
- Modular Robots and Swarm Intelligence
- Fluorine in Organic Chemistry
- Advanced Physical and Chemical Molecular Interactions
- Molecular Sensors and Ion Detection
The University of Sydney
2009-2024
Westmead Hospital
2016-2024
Liverpool Hospital
2022-2024
New South Wales Department of Health
2024
Amyloidosis Foundation
2024
Alnylam Pharmaceuticals (United States)
2023
University of South Alabama
2007-2023
Prince of Wales Hospital
2022-2023
UNSW Sydney
2022-2023
Inserm
2023
The study objective was to assess the effect of vutrisiran, an RNA interference therapeutic that reduces transthyretin (TTR) production, in patients with hereditary (ATTRv) amyloidosis polyneuropathy.HELIOS-A a phase 3, global, open-label comparing efficacy and safety vutrisiran external placebo group (APOLLO study). Patients were randomized 3:1 subcutaneous 25 mg every 3 months (Q3M) or intravenous patisiran 0.3 mg/kg weeks (Q3W) for 18 months.HELIOS-A enrolled 164 (vutrisiran, n = 122;...
Transthyretin amyloidosis, also called ATTR is associated with accumulation of amyloid deposits in the heart and commonly manifests as progressive cardiomyopathy. Patisiran, an RNA interference therapeutic agent, inhibits production hepatic transthyretin. Download a PDF Research Summary. In this phase 3, double-blind, randomized trial, we assigned patients hereditary, known variant, or wild-type cardiac 1:1 ratio, to receive patisiran (0.3 mg per kilogram body weight) placebo once every 3...
Arrays of octameric peptide libraries on cellulose paper were screened by using 32 P-autophosphorylated cGMP-dependent protein kinase Iα (cGPK) to identify sequences with high binding affinity for cGPK. Iterative deconvolution every amino acid position in the peptides identified sequence LRK 5 H (W45) as having highest affinity. Binding W45 cGPK resulted selective inhibition K i values 0.8 μM and 560 cAMP-dependent (cAPK), respectively. Fusion membrane translocation signals from HIV-1 tat...
Cardiac amyloidosis (CA) is an under-recognized cause of heart failure. Left atrial (LA) myopathy contributes to a worse prognosis in failure and feature transthyretin (ATTR) light-chain (AL) CA. LA mechanical dispersion (LA-MD) novel marker intra-atrial dyssynchrony implicated the future development fibrillation (AF).
Quantum chemistry computations have been used to investigate hydrogen-atom abstraction by chlorine atom from protonated and N-acetylated amino acids. The results are consistent with the decreased reactivity at backbone α-carbon adjacent side-chain positions that is observed experimentally. individual effects of NH(3)(+), COOH, NHAc substituents examined reveal important insights. NH(3)(+) group in isolation found be deactivating α-position, while acetamido activating. For COOH group, polar...
Allogeneic hematopoietic stem cell transplantation (HSCT) can lead to significant changes the HIV reservoir and immune responses, indicating that further characterization of HIV-infected patients undergoing HSCT is warranted.We studied 3 who underwent after either reduced intensity conditioning or myeloablative regimen. We measured antigens antibodies (Ag/Ab), HIV-specific CD4 T-cell RNA, DNA in plasma, peripheral blood mononuclear cells, isolated T cells from blood, lymph node cells. The...
Mechanisms regulating uterine contractility are poorly understood. We hypothesized that a specific isoform of small conductance Ca 2+ -activated K + (SK) channel, SK3, promotes feedback regulation myometrial and hence relaxation the uterus. To determine functional impact SK3 channels, we assessed isometric contractions strips from genetically altered mice (SK3 T/T ), in which is overexpressed can be suppressed by oral administration doxycycline +Dox). found protein mouse myometrium, this...
We determined the diseases associated with extremely high levels of alkaline phosphatase in hospitalized patients. Computerized laboratory records Hospital Saint Raphael identified all inpatients who had elevations above 1,000 U/l from April 1994 to September 1995. Thirty-seven were identified. Six bone involvement malignancy or Paget's disease and eliminated further analysis, 31 patients included study. Levels ranged 1,014 3,360 U/l. Ten sepsis as cause elevated phosphatase. These...
The short-time nuclear dynamics of Cu(H(2)O) is investigated using femtosecond photodetachment-photoionization spectroscopy and time-dependent quantum wave packet calculations. initiated in the electronic ground state complex by electron photodetachment from Cu(-)(H(2)O) complex, where hydrogen atoms are oriented toward Cu. Several time-resolved resonant multiphoton ionization schemes used to probe ensuing reorientation dissociation. Immediately following photodetachment, neutral far its...
An ab initio investigation of the potential energy surfaces and vibrational energies wave functions anion, neutral, cation Cu(H(2)O) complexes is presented. The equilibrium geometries harmonic frequencies three charge states are calculated at MP2 level theory. CCSD(T) calculations predict a vertical electron detachment for anion complex 1.65 eV ionization neutral 6.27 eV. Potential copper-water complexes. These used in variational from these, dissociation D(0) predicted to be 0.39, 0.16,...
Differentiating phenotypes of cardiac "hypertrophy" characterised by increased wall thickness on echocardiography is essential for management and prognostication. Transthoracic the most commonly used screening test this purpose. We sought to identify echocardiographic markers that distinguish infiltrative storage disorders present with left ventricular (LV) thickness, namely, amyloidosis (CA) Anderson-Fabry disease (AFD), from hypertensive heart (HHT).Patients were retrospectively recruited...
The heats of formation four C2H3N molecules and the three most stable C2H2N cations, namely, cyanomethyl, isocyanomethyl, azirinyl, together with their corresponding radicals anions, have been calculated at G2, G2(//QCI), CBS-Q, CBS-RAD levels theory. In addition, bond dissociation enthalpies gas-phase acidities electron affinities ionization energies free derived. Comparison theoretical results available experimental values shows generally good agreement directly measured data but...
A transverse plantar incision for fascial release was assessed pain relief, numbness, and subsequent heel pad symptoms. Twenty-seven feet in 26 patients who underwent fascia were reviewed with a minimum follow-up of 2 years after surgery (average, 37.6 months). Comprehensive data obtained on 25 (24 patients) (93% response rate). The origin completely transected all cases. This led to complete resolution symptoms 19 residual minor six feet. After years, four had developed recurrent symptoms,...
A high-level quantum chemistry investigation has been carried out for the abstraction by chlorine atom of hydrogen from methane and five monosubstituted methanes, chosen to reflect chemical functionalities contained in amino acids peptides. modified W1′ procedure is used calculate benchmark barriers reaction energies six reactions. The reactions demonstrate a broad range barrier heights energies, which can be rationalized using curve-crossing molecular orbital theory models. In addition,...
The ultrafast relaxation dynamics of Cu(H(2)O)(2) is investigated using femtosecond photodetachment-photoionization spectroscopy. In addition, stationary points on the anion, neutral, and cation potential energy surfaces are characterized by ab initio electronic structure calculations. Electron photodetachment from Cu(-)(H(2)O)(2) initiates ground-state surface neutral Cu(H(2)O)(2). resulting complexes experience large-amplitude H(2)O reorientation dissociation. time evolution fragmentation...
Cardiac involvement in AL amyloidosis portends a poor prognosis. 2D-speckle tracking echocardiography (2D-STE) strain can identify subclinical cardiac involvement. This study performed multilayer and multiplanar 2D-STE myocardial analysis.We compared 75 patients to 49 hypertensive healthy controls. Longitudinal was obtained from epicardial, mid-myocardial endocardial layers; segmental measured basal, mid apical segments.Global longitudinal reduced epicardial (-14.3 ± -4.0% vs. -17.4 2.2%...
Ultrafast pump-probe studies of I2- recombination in size-selected (CO2)n cluster ions demonstrate long time coherent motions clusters and the resulting non-statistical energy flow cluster. For photodissociated to produce either I- + I or I*, we identify a solvent-driven transfer process without condensed phase counterpart. The mechanism involved is Marcus-like curve crossing, with driving force arising from asymmetric solvation, not just solvent orientation. By substituting another halogen...
We present a series of five cases who presented to our institution with treatment-refractory mucosal ulceration, all whom were subsequently diagnosed paraneoplastic pemphigus (PNP). This case highlights the diagnostic and treatment considerations for PNP - in particular, steroid-dependent, recalcitrant, polymorphic manifestations; combination histopathological clinical findings that may overlap clinically similar diseases, example, vulgaris lichen planus; importance immunopathological its...