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News and Views | 28 July 2014
Something wicked this way comes: huntingtin
- Albert R La Spada
Nature Neuroscience 17 , 1014–1015

Rights & permissionsfor article Something wicked this way comes: huntingtin . Opens in a new window.
1 March 2009
Getting a handle on Huntington's disease: silencing neurodegeneration
- ...caused by that gene). Albert R. La Spada examines efforts to target bad...
- Albert R La Spada
Nature Medicine 15 , 252–253

Rights & permissionsfor article Getting a handle on Huntington's disease: silencing neurodegeneration . Opens in a new window.
News and Views | 6 January 2012
Finding a sirtuin truth in Huntington's disease
- Albert R La Spada
Nature Medicine 18 , 24–26

Rights & permissionsfor article Finding a sirtuin truth in Huntington's disease . Opens in a new window.
News and Views | 1 December 2009
Memantine strikes the perfect balance
- Albert R La Spada
Nature Medicine 15 , 1355–1356

Rights & permissionsfor article Memantine strikes the perfect balance . Opens in a new window.
News and Views | 1 October 2005
Targeting toxic proteins for turnover
- Albert R La Spada
- & Patrick Weydt
Nature Medicine 11 , 1052–1053

Rights & permissionsfor article Targeting toxic proteins for turnover . Opens in a new window.
Research | 13 April 2020
Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72
- Qiang Zhu
- , Jie Jiang
- , Tania F. Gendron
- , Melissa McAlonis-Downes
- , Lulin Jiang
- , Amy Taylor
- , Sandra Diaz Garcia
- , Somasish Ghosh Dastidar
- , Maria J. Rodriguez
- , Patrick King
- , Yongjie Zhang
- , Albert R. La Spada
- , Huaxi Xu
- , Leonard Petrucelli
- , John Ravits
- , Sandrine Da Cruz
- , Clotilde Lagier-Tourenne
- & Don W. Cleveland
Nature Neuroscience 23 , 615–624

Rights & permissionsfor article Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in <i>C9orf72</i> . Opens in a new window.
Research | 1 December 1992
Meiotic stability and genotype – phenotype correlation of the trinucleotide repeat in X–linked spinal and bulbar muscular atrophy
Nature Genetics 2 , 301–304

Rights & permissionsfor article Meiotic stability and genotype – phenotype correlation of the trinucleotide repeat in X–linked spinal and bulbar muscular atrophy . Opens in a new window.
Research | 4 July 1991
Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy
Nature 352 , 77–79

Rights & permissionsfor article Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy . Opens in a new window.
News and Views | 1 July 2004
Dynamic mutations on the move in Banff
Nature Genetics 36 , 667–670

Rights & permissionsfor article Dynamic mutations on the move in Banff . Opens in a new window.
Research | 5 March 2019 | Open
Gene expression analysis reveals early dysregulation of disease pathways and links Chmp7 to pathogenesis of spinal and bulbar muscular atrophy
Scientific Reports 9 , 1–16

Rights & permissionsfor article Gene expression analysis reveals early dysregulation of disease pathways and links <i>Chmp7</i> to pathogenesis of spinal and bulbar muscular atrophy . Opens in a new window.
Reviews | 26 September 2018
Converging pathways in neurodegeneration, from genetics to mechanisms
Nature Neuroscience 21 , 1300–1309

Rights & permissionsfor article Converging pathways in neurodegeneration, from genetics to mechanisms . Opens in a new window.
Research | 5 March 2018
Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy
- Nisha M Badders
- , Ane Korff
- , Helen C Miranda
- , Pradeep K Vuppala
- , Rebecca B Smith
- , Brett J Winborn
- , Emmanuelle R Quemin
- , Bryce L Sopher
- , Jennifer Dearman
- , James Messing
- , Nam Chul Kim
- , Jennifer Moore
- , Brian D Freibaum
- , Anderson P Kanagaraj
- , Baochang Fan
- , Heather Tillman
- , Ping-Chung Chen
- , Yingzhe Wang
- , Burgess B Freeman III
- , Yimei Li
- , Hong Joo Kim
- , Albert R La Spada
- & J Paul Taylor
Nature Medicine 24 , 427–437

Rights & permissionsfor article Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy . Opens in a new window.
Research | 5 March 2018 | Open
MAP4K3 mediates amino acid-dependent regulation of autophagy via phosphorylation of TFEB
- Cynthia L. Hsu
- , Elian X. Lee
- , Kara L. Gordon
- , Edwin A. Paz
- , Wen-Chuan Shen
- , Kohta Ohnishi
- , Jill Meisenhelder
- , Tony Hunter
- & Albert R. La Spada
Nature Communications 9 , 1–15

Rights & permissionsfor article MAP4K3 mediates amino acid-dependent regulation of autophagy via phosphorylation of TFEB . Opens in a new window.
Research | 7 December 2015
PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically
- Audrey S Dickey
- , Victor V Pineda
- , Taiji Tsunemi
- , Patrick P Liu
- , Helen C Miranda
- , Stephen K Gilmore-Hall
- , Nicole Lomas
- , Kunal R Sampat
- , Anne Buttgereit
- , Mark-Joseph Manalang Torres
- , April L Flores
- , Martin Arreola
- , Nicolas Arbez
- , Sergey S Akimov
- , Terry Gaasterland
- , Eduardo R Lazarowski
- , Christopher A Ross
- , Gene W Yeo
- , Bryce L Sopher
- , Gavin K Magnuson
- , Anthony B Pinkerton
- , Eliezer Masliah
- & Albert R La Spada
Nature Medicine 22 , 37–45

Rights & permissionsfor article PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically . Opens in a new window.
Research | 10 August 2014
Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA
- Constanza J Cortes
- , Helen C Miranda
- , Harald Frankowski
- , Yakup Batlevi
- , Jessica E Young
- , Amy Le
- , Nishi Ivanov
- , Bryce L Sopher
- , Cassiano Carromeu
- , Alysson R Muotri
- , Gwenn A Garden
- & Albert R La Spada
Nature Neuroscience 17 , 1180–1189

Rights & permissionsfor article Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA . Opens in a new window.
Research | 7 May 2013
A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases
- Kyota Fujita
- , Yoko Nakamura
- , Tsutomu Oka
- , Hikaru Ito
- , Takuya Tamura
- , Kazuhiko Tagawa
- , Toshikazu Sasabe
- , Asuka Katsuta
- , Kazumi Motoki
- , Hiroki Shiwaku
- , Masaki Sone
- , Chisato Yoshida
- , Masahisa Katsuno
- , Yoshinobu Eishi
- , Miho Murata
- , J. Paul Taylor
- , Erich E. Wanker
- , Kazuteru Kono
- , Satoshi Tashiro
- , Gen Sobue
- , Albert R. La Spada
- & Hitoshi Okazawa
Nature Communications 4 , 1–13

Rights & permissionsfor article A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases . Opens in a new window.
Research | 27 August 2006
Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport
- Sara K Custer
- , Gwenn A Garden
- , Nishi Gill
- , Udo Rueb
- , Randell T Libby
- , Christian Schultz
- , Stephan J Guyenet
- , Thomas Deller
- , Lesnick E Westrum
- , Bryce L Sopher
- & Albert R La Spada
Nature Neuroscience 9 , 1302–1311

Rights & permissionsfor article Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport . Opens in a new window.
Reviews | 23 February 2010
Repeat expansion disease: progress and puzzles in disease pathogenesis
- Albert R. La Spada
- & J. Paul Taylor
Nature Reviews Genetics 11 , 247–258

Rights & permissionsfor article Repeat expansion disease: progress and puzzles in disease pathogenesis . Opens in a new window.

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Something wicked this way comes: huntingtin

Getting a handle on Huntington's disease: silencing neurodegeneration

Finding a sirtuin truth in Huntington's disease

Memantine strikes the perfect balance

Targeting toxic proteins for turnover

Reduced C9ORF72 function exacerbates gain of toxicity from ALS/FTD-causing repeat expansion in C9orf72

Meiotic stability and genotype – phenotype correlation of the trinucleotide repeat in X–linked spinal and bulbar muscular atrophy

Androgen receptor gene mutations in X-linked spinal and bulbar muscular atrophy

Dynamic mutations on the move in Banff

Gene expression analysis reveals early dysregulation of disease pathways and links Chmp7 to pathogenesis of spinal and bulbar muscular atrophy

Converging pathways in neurodegeneration, from genetics to mechanisms

Selective modulation of the androgen receptor AF2 domain rescues degeneration in spinal bulbar muscular atrophy

MAP4K3 mediates amino acid-dependent regulation of autophagy via phosphorylation of TFEB

PPAR-δ is repressed in Huntington's disease, is required for normal neuronal function and can be targeted therapeutically

Polyglutamine-expanded androgen receptor interferes with TFEB to elicit autophagy defects in SBMA

A functional deficiency of TERA/VCP/p97 contributes to impaired DNA repair in multiple polyglutamine diseases

Bergmann glia expression of polyglutamine-expanded ataxin-7 produces neurodegeneration by impairing glutamate transport

Repeat expansion disease: progress and puzzles in disease pathogenesis

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