A mutation in the amyloid precursor protein associated with hereditary Alzheimer's disease

J Murrell, M Farlow, B Ghetti, MD Benson - Science, 1991 - science.org
Alzheimer's disease is a form of localized amyloidosis characterized by cerebral cortical
amyloid plaques, neurofibrillary tangles, and amyloid deposits within the walls of …

[HTML][HTML] Inotersen treatment for patients with hereditary transthyretin amyloidosis

MD Benson, M Waddington-Cruz, JL Berk… - … England Journal of …, 2018 - Mass Medical Soc
Background Hereditary transthyretin amyloidosis is caused by pathogenic single-nucleotide
variants in the gene encoding transthyretin (TTR) that induce transthyretin misfolding and …

Amyloid fibril proteins and amyloidosis: chemical identification and clinical classification International Society of Amyloidosis 2016 Nomenclature Guidelines

JD Sipe, MD Benson, JN Buxbaum, S Ikeda, G Merlini… - Amyloid, 2016 - Taylor & Francis
Full article: Amyloid fibril proteins and amyloidosis: chemical identification and clinical
classification International Society of Amyloidosis 2016 Nomenclature Guidelines Skip to Main …

Nomenclature 2014: Amyloid fibril proteins and clinical classification of the amyloidosis

JD Sipe, MD Benson, JN Buxbaum, S Ikeda, G Merlini… - Amyloid, 2014 - Taylor & Francis
Background When the chemical diversity of amyloid and amyloidosis became apparent in the
mid-1970s, participants in the International Symposium on Amyloidosis, Helsinki, Finland, …

Amyloid nomenclature 2018: recommendations by the International Society of Amyloidosis (ISA) nomenclature committee

MD Benson, JN Buxbaum, DS Eisenberg, G Merlini… - Amyloid, 2018 - Taylor & Francis
The nomenclature committee of the International Society of Amyloidosis (ISA) meets every
second year to discuss and formulate recommendations. The conclusions from the discussion …

Amyloid nomenclature 2020: update and recommendations by the International Society of Amyloidosis (ISA) nomenclature committee

MD Benson, JN Buxbaum, DS Eisenberg, G Merlini… - Amyloid, 2020 - Taylor & Francis
The ISA Nomenclature Committee met electronically before and directly after the XVII ISA
International Symposium on Amyloidosis, which, unfortunately, had to be virtual in September …

The molecular biology and clinical features of amyloid neuropathy

MD Benson, JC Kincaid - Muscle & Nerve: Official Journal of …, 2007 - Wiley Online Library
Neuropathy is often a major manifestation of systemic amyloidosis. It is most frequently seen
in patients with hereditary transthyretin (TTR) amyloidosis, but is also present in 20% of …

Amyloid fibril protein nomenclature: 2010 recommendations from the nomenclature committee of the International Society of Amyloidosis

JD Sipe, MD Benson, JN Buxbaum, SI Ikeda, G Merlini… - Amyloid, 2010 - Taylor & Francis
A system of amyloid fibril nomenclature based on the chemical identity of the amyloid fibril
forming protein is recommended. This system has been in use for approximately 40 years, but …

The protofilament substructure of amyloid fibrils

LC Serpell, M Sunde, MD Benson, GA Tennent… - Journal of molecular …, 2000 - Elsevier
Tissue deposition of normally soluble proteins, or their fragments, as insoluble amyloid fibrils
causes the usually fatal, acquired and hereditary systemic amyloidoses and is associated …

A primer of amyloid nomenclature

P Westermark, MD Benson, JN Buxbaum, AS Cohen… - Amyloid, 2007 - Taylor & Francis
The increasing knowledge of the exact biochemical nature of the localized and systemic
amyloid disorders has made a logical and easily understood nomenclature absolutely …