Results 71 to 80 of about 16,704,778 (257)

A novel hotspot of gelsolin instability and aggregation propensity triggers a new mechanism of amyloidosis [PDF]

open access: yesarXiv, 2021
The multidomain protein gelsolin (GSN) is composed of six homologous modules, sequentially named G1 to G6. Single point substitutions in this protein are responsible for AGel amyloidosis, a hereditary disease characterized by progressive corneal lattice dystrophy, cutis laxa, and polyneuropathy. Several different amyloidogenic variants of GSN have been
arxiv  

Citrate stabilized gold nanoparticles interfere with amyloid fibril formation: D76N and ΔN6 \b{eta}2-microglobulin variants [PDF]

open access: yesNanoscale, 2018, 10, 4793, 2018
Protein aggregation including the formation of dimers and multimers in solution, underlies an array of human diseases such as systemic amyloidosis which is a fatal disease caused by misfolding of native globular proteins damaging the structure and function of affected organs.
arxiv   +1 more source

Electronic Structure of ThPd$_2$Al$_3$: an impact of the U $5f$ states in the electronic structure of UPd$_2$Al$_3$ [PDF]

open access: yes, 2022
The electronic structure of ThPd$_2$Al$_3$, which is isostructural to the heavy fermion superconductor UPd$_2$Al$_3$, was investigated by photoelectron spectroscopy. The band structure and Fermi surfaces of ThPd$_2$Al$_3$ were obtained by angle-resolved photoelectron spectroscopy (ARPES), and the results were well-explained by the band-structure ...
arxiv   +1 more source

Recurrent syncope and cardiac arrest in a patient with systemic light chain amyloidosis treated with bortezomib

open access: yesHematology Reports, 2016
About 10-15% of patients with multiple myeloma develop light chain (AL) amyloidosis. AL amyloidosis is a systemic disease that may involve multiple organs, often including the heart. It may present clinically with bradyarrhythmia and syncope.
Navin Jaipaul   +2 more
doaj   +1 more source

Treatment patterns and outcomes in light chain amyloidosis: An institutional registry of amyloidosis report in Argentina.

open access: yesPLoS ONE, 2022
Light chain (AL) amyloidosis is a form of systemic amyloidosis, causing organ dysfunction, mainly affecting the heart and kidney. Patient-tailored and risk-adapted decision making is critical in AL amyloidosis management.
Maria Lourdes Posadas-Martinez   +7 more
doaj   +1 more source

The structure of N184K amyloidogenic variant of gelsolin highlights the role of the H-bond network for protein stability and aggregation properties [PDF]

open access: yesEur Biophys J. 2019 Nov 13, 2019
Mutations in the gelsolin protein are responsible for a rare conformational disease known as AGel amyloidosis. Four of these mutations are hosted by the second domain of the protein (G2): D187N/Y, G167R and N184K. The impact of the latter has been so far evaluated only by studies on the isolated G2.
arxiv   +1 more source

Upfront CyBorD in AL amyloidosis [PDF]

open access: yesBlood, 2015
In this issue of Blood , [Palladini et al][1] report on the outcome of a large series of 230 patients with systemic immunoglobulin (Ig) amyloid light chain (AL) amyloidosis treated frontline with cyclophosphamide, bortezomib, and dexamethasone (CyBorD) at 2 referral centers.[1][2] ![Figure][3]
M. Teresa Cibeira, Joan Bladé
openaire   +3 more sources

Kidney-limited AL amyloidosis: a case report and review of the literature

open access: yesJournal of Community Hospital Internal Medicine Perspectives, 2021
Amyloidosis involves the deposition of abnormal proteins in various tissues and results in progressive organ dysfunction, commonly affecting multiple organs.
Sara Velayati   +5 more
doaj   +1 more source

Beyond Survival in AL amyloidosis: Identifying and Satisfying Patients’ Needs

open access: yesHemato, 2022
The survivorship needs of patients with light-chain (AL) amyloidosis are complex, as is the diagnosis and treatment itself. Early diagnosis is critical in improving patient outcomes; however, given the nonspecific nature of the symptoms, most patients ...
Hamza Hassan, Vaishali Sanchorawala
doaj   +1 more source

An unusual case of chronic lymphocytic leukemia, multiple myeloma and cardiac amyloidosis

open access: yesJournal of Community Hospital Internal Medicine Perspectives, 2017
Light chain amyloidosis has very rarely been reported in association with chronic lymphocytic leukemia (CLL). We reported on a 76-years-old female who presented with simultaneous kappa-restricted chronic lymphocytic leukemia (CLL) and a lambda-restricted
Dongyan Liu   +2 more
doaj   +1 more source

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