scholarly journals Intrathecal delivery of frataxin mRNA encapsulated in lipid nanoparticles to dorsal root ganglia as a potential therapeutic for Friedreich’s ataxia

2016 ◽  
Vol 6 (1) ◽  
Author(s):  
Joseph F. Nabhan ◽  
Kristy M. Wood ◽  
Varada P. Rao ◽  
Jeffrey Morin ◽  
Surya Bhamidipaty ◽  
...  
Keyword(s):  
Dorsal Root Ganglia ◽  
Dorsal Root ◽  
Friedreich’S Ataxia ◽  
Lipid Nanoparticles ◽  
Friedreich's Ataxia

scholarly journals Frataxin gene editing rescues Friedreich’s ataxia pathology in dorsal root ganglia organoid-derived sensory neurons

2020 ◽  
Vol 11 (1) ◽  
Author(s):  
Pietro Giuseppe Mazzara ◽  
Sharon Muggeo ◽  
Mirko Luoni ◽  
Luca Massimino ◽  
Mattia Zaghi ◽  
...  
Keyword(s):  
Sensory Neurons ◽  
Dorsal Root Ganglia ◽  
Dorsal Root ◽  
Gene Editing ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Frataxin Gene

scholarly journals Progressive gaa expansions in dorsal root ganglia of Friedreich's ataxia patients

2007 ◽  
Vol 61 (1) ◽  
pp. 55-60 ◽  
Author(s):  
Irene De Biase ◽  
Astrid Rasmussen ◽  
Dan Endres ◽  
Sahar Al-Mahdawi ◽  
Antonella Monticelli ◽  
...  
Keyword(s):  
Dorsal Root Ganglia ◽  
Dorsal Root ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia

scholarly journals Frataxin Deficiency Leads to Defects in Expression of Antioxidants and Nrf2 Expression in Dorsal Root Ganglia of the Friedreich's Ataxia YG8R Mouse Model

2013 ◽  
Vol 19 (13) ◽  
pp. 1481-1493 ◽  
Author(s):  
Yuxi Shan ◽  
Robert A. Schoenfeld ◽  
Genki Hayashi ◽  
Eleonora Napoli ◽  
Tasuku Akiyama ◽  
...  
Keyword(s):  
Mouse Model ◽  
Dorsal Root Ganglia ◽  
Dorsal Root ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Nrf2 Expression

scholarly journals Stem Cells from Wildtype and Friedreich’s Ataxia Mice Present Similar Neuroprotective Properties in Dorsal Root Ganglia Cells

PLoS ONE ◽  
2013 ◽  
Vol 8 (5) ◽  
pp. e62807 ◽  
Author(s):  
Jonathan Jones ◽  
Alicia Estirado ◽  
Carolina Redondo ◽  
Salvador Martinez
Keyword(s):  
Stem Cells ◽  
Dorsal Root Ganglia ◽  
Dorsal Root ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia

Dorsal root ganglion proteins in Friedreich's ataxia

1993 ◽  
Vol 163 (2) ◽  
pp. 182-184 ◽  
Author(s):  
J.R. Small ◽  
P.K. Thomas ◽  
A.H.V. Schapira
Keyword(s):  
Dorsal Root Ganglion ◽  
Dorsal Root ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia

scholarly journals Ultrastructural Observations on Spinal Ganglion Biopsy in Friedreich’s Ataxia: A Preliminary Report

1982 ◽  
Vol 9 (2) ◽  
pp. 137-139 ◽  
Author(s):  
J. Lamarche ◽  
C. Luneau ◽  
B. Lemieux
Keyword(s):  
Dorsal Root Ganglion ◽  
Dorsal Root ◽  
Preliminary Report ◽  
Surgical Intervention ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia ◽  
Spinal Ganglion ◽  
Apparent Decrease ◽  
Preliminary Study ◽  
Axonal Swellings

ABSTRACT:We report the preliminary study of a dorsal root ganglion obtained at biopsy during a surgical intervention in a patient with Friedreich’s ataxia. There was an apparent decrease in the number of large myelinated fibers without necrosis, but with numerous axonal swellings consisting mainly of dense accumulated neurofilaments. Large amounts of lipofuscin were also found as well as some onion bulb formations suggesting a process of axonal atrophy.

The dorsal root ganglion in Friedreich’s ataxia

2009 ◽  
Vol 118 (6) ◽  
pp. 763-776 ◽  
Author(s):  
Arnulf H. Koeppen ◽  
Jennifer A. Morral ◽  
Ashley N. Davis ◽  
Jiang Qian ◽  
Simone V. Petrocine ◽  
...  
Keyword(s):  
Dorsal Root Ganglion ◽  
Dorsal Root ◽  
Friedreich’S Ataxia ◽  
Friedreich's Ataxia

Membrane-associated microtubular areays induced in proximal myelinated processes of dorsal root ganglia by large doses of vitamin B6

1986 ◽  
Vol 44 ◽  
pp. 368-369
Author(s):  
V.J. Montpetit ◽  
S. Dancea ◽  
L. Tryphonas ◽  
D.F. Clapin
Keyword(s):  
Animal Model ◽  
Endoplasmic Reticulum ◽  
Dorsal Root Ganglia ◽  
Rough Endoplasmic Reticulum ◽  
Dorsal Root ◽  
Vitamin B6 ◽  
Morphological Changes ◽  
Model System ◽  
Sensory Nerves ◽  
Peripheral Sensory

Very large doses of pyridoxine (vitamin B6) are neurotoxic in humans, selectively affecting the peripheral sensory nerves. We have undertaken a study of the morphological and biochemical aspects of pyridoxine neurotoxicity in an animal model system. Early morphological changes in dorsal root ganglia (DRG) associated with pyridoxine megadoses include proliferation of neurofilaments, ribosomes, rough endoplasmic reticulum, and Golgi complexes. We present in this report evidence of the formation of unique aggregates of microtubules and membranes in the proximal processes of DRG which are induced by high levels of pyridoxine.

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