Results 91 to 100 of about 319,403 (201)

Proteostasis of organelles in aging and disease

The FEBS Journal, Volume 293, Issue 18, Page 5600-5626, September 2026.
Cells rely on regulated proteostasis mechanisms to keep their internal compartments functioning properly. When these mechanisms fail, damaged proteins accumulate, disrupting organelles, such as the nucleus, mitochondria, endoplasmic reticulum, Golgi, and lysosomes, as well as membraneless organelles, such as stress granules, processing bodies, the ...
Yara Nabawi, Cansu Doğan, Dunja Petrović, Gülce Perçin, Seda Koyuncu, David Vilchez   +5 more
wiley   +1 more source

VRK1‐Related Motor Neuropathy With Upper Motor Neuron Signs and Selective Muscle Involvement

Journal of the Peripheral Nervous System, Volume 31, Issue 3, September 2026.
ABSTRACT Introduction Hereditary motor neuropathies (HMN) represent a heterogeneous group of disorders with wide clinical and genetic variability. Despite advances in molecular diagnostics, approximately 50% of cases remain genetically unresolved, particularly those where distinguishing length‐dependent motor neuropathy from motor neuron disorder with ...
Manoella Guerra de Albuquerque Bueno, Diogo Fernandes dos Santos, Alexander M. Rossor, Matilde Laura, Alejandro Horga, Rodrigo Siqueira Soares Frezatti, Eduardo Vaz de Sousa Ferreira, Julian C. Blake, Jasper M. Morrow, Mary M. Reilly, Wilson Marques Júnior, Pedro José Tomaselli   +11 more
wiley   +1 more source

Molecular Mediators Associated With Myelination, Demyelination, and Remyelination in the Peripheral Nervous System

Journal of the Peripheral Nervous System, Volume 31, Issue 3, September 2026.
ABSTRACT The peripheral nervous system (PNS) is responsible for innervating all regions of the body outside of the central nervous system (CNS), the latter consisting of the brain, spinal cord, and optic nerves. While myelin is an essential component for the efficient functioning of both CNS and peripheral nerve cells, it is particularly important for ...
Kathleen Margaret Hagen, Shalina Sheryl Ousman   +1 more
wiley   +1 more source

Familial Amyloid Neuropathy [PDF]

, 2020
openaire   +1 more source

Impact of Baseline Polyneuropathy Severity on Eplontersen Efficacy in the NEURO‐TTRansform Clinical Trial

Journal of the Peripheral Nervous System, Volume 31, Issue 3, September 2026.
ABSTRACT Background and Aims In the NEURO‐TTRansform clinical trial (NCT04136184), eplontersen improved neuropathy impairment and quality of life (QoL) through Week 66 versus the NEURO‐TTR historical placebo in patients with hereditary transthyretin amyloidosis with polyneuropathy (ATTRv‐PN).
Thomas H. Brannagan III, Markus Weiler, Márcia Waddington Cruz, Sami Khella, John L. Berk, Morie Gertz, Ahmad Masri, Mathew S. Maurer, Jonas Wixner, Jonatan Nåtman, T. Jesse Kwoh, Maksym Pola, Laura Obici   +12 more
wiley   +1 more source

Reply to “Sural/Radial Amplitude Ratio in Non‐Length‐Dependent Neuropathy (NLDN): A Call for More Standardized Methodology and Further Evaluation of Distal NLDN”

Muscle &Nerve, Volume 74, Issue 3, Page 761-762, September 2026.
Antoine Pegat, Guillaume Fargeot, Françoise Bouhour   +2 more
wiley   +1 more source

Cryptogenic Neuropathy - a retrospective analysis - Transthyretin-related familial amyloid polyneuropathy - differential diagnostic pathway [PDF]

Neurologie pro praxi, 2020
Jana Junkerová, Eva Kovalová
openaire   +1 more source

Structural Studies of Cystatin B Amyloid Fibre and Oligomer [PDF]

, 2013
Amyloid fibres are characteristic of over 25 degenerative human diseases including Alzheimer’s and Parkinson’s disease. Amyloid fibres are insoluble, highly stable, ordered cross-β sheet structures, which form as a result of conformational change and ...
Davis, Peter J.
core  

Familial Amyloid Neuropathy associated with A-V Block

Japanese Journal of National Medical Services, 1977
ANZAI, Nobuyuki, IRIYAMA, Tadashi, YAMADA, Manabu, ISHIHARA, Shigeki, KITO, Shozo, ITOGA, Eiko   +5 more
openaire   +1 more source

The Red Flags Were Missing, the Indication Was Not: Genetic Testing in Heart Failure and Cardiomyopathy. [PDF]

Methodist Debakey Cardiovasc J
Bakr Salama A.
europepmc   +1 more source