Zobrazeno 1 - 10
of 34
pro vyhledávání: '"Benoit J. Gentil"'
Autor:
Doris Lou Demy, Maria Letizia Campanari, Raphael Munoz-Ruiz, Heather D. Durham, Benoit J. Gentil, Edor Kabashi
Publikováno v:
Cells, Vol 9, Iss 5, p 1238 (2020)
Neurofilaments (NFs), a major cytoskeletal component of motor neurons, play a key role in the differentiation, establishment and maintenance of their morphology and mechanical strength. The de novo assembly of these neuronal intermediate filaments re
Externí odkaz:
https://doaj.org/article/d61295b1c8d9429d9fb15f252790ddec
Autor:
Afrooz Dabbaghizadeh, Alexandre Paré, Zacharie Cheng-Boivin, Robin Dagher, Sandra Minotti, Marie-Josée Dicaire, Bernard Brais, Jason C. Young, Heather D. Durham, Benoit J. Gentil
Publikováno v:
International Journal of Molecular Sciences; Volume 23; Issue 24; Pages: 15742
Autosomal Recessive Spastic Ataxia of the Charlevoix Saguenay (ARSACS) is caused by mutation in the SACS gene resulting in loss of function of the protein sacsin. A key feature is the formation of abnormal bundles of neurofilaments (NF) in neurons an
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::ace0c6c40b8ef42b7a753a6ceb5f935c
Autor:
Jacques Baudier, Benoit J Gentil
Publikováno v:
Biomolecules, Vol 10, Iss 6, p 843 (2020)
In mammals, adipose tissue is an active secretory tissue that responds to mild hypothermia and as such is a genuine model to study molecular and cellular adaptive responses to cold-stress. A recent study identified a mammal-specific protein of the en
Externí odkaz:
https://doaj.org/article/a42c9ff1db864d57a501bf0fe65b6dc4
Autor:
Young Jc, Bernard B, Benoit J. Gentil, Dicaire M, Sandra Minotti, Dabbaghizadeh A, Dagher R, Cheng-Boivin Z, Heather D. Durham, Paré A
Autosomal Recessive Spastic Ataxia of the Charlevoix Saguenay (ARSACS), is caused by loss of function mutations in the SACS gene, which encodes sacsin, a giant protein of 520 kDa. A key feature of the absence of sacsin in cells is the formation of ab
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::a22f7b7e83a0faccc72f1212d0a077be
https://doi.org/10.1101/2021.07.14.452331
https://doi.org/10.1101/2021.07.14.452331
Publikováno v:
Molecular genetics and metabolism. 133(1)
Mutations in the HADHB gene lead to Mitochondrial Trifunctional Protein (MTP) deficiency. MTP deficiency is a rare autosomal recessive disorder affecting long-chain fatty acid oxidation. Patients affected by MTP deficiency are unable to metabolize lo
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fb723e882d356a9eac8cdff294c7a44b
https://pubmed.ncbi.nlm.nih.gov/33744096
https://pubmed.ncbi.nlm.nih.gov/33744096
Autor:
Benoit J. Gentil, María Letizia Campanari, Edor Kabashi, Raphael Munoz-Ruiz, Doris Lou Demy, Heather D. Durham
Publikováno v:
Cells
Cells, MDPI, 2020, 9 (5), pp.1238. ⟨10.3390/cells9051238⟩
Cells, Vol 9, Iss 1238, p 1238 (2020)
Cells, 2020, 9 (5), pp.1238. ⟨10.3390/cells9051238⟩
Volume 9
Issue 5
Cells, MDPI, 2020, 9 (5), pp.1238. ⟨10.3390/cells9051238⟩
Cells, Vol 9, Iss 1238, p 1238 (2020)
Cells, 2020, 9 (5), pp.1238. ⟨10.3390/cells9051238⟩
Volume 9
Issue 5
Neurofilaments (NFs), a major cytoskeletal component of motor neurons, play a key role in the differentiation, establishment and maintenance of their morphology and mechanical strength. The de novo assembly of these neuronal intermediate filaments re
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3f38faab1820ac53b9230ec91a26e3b8
https://hal.sorbonne-universite.fr/hal-03152405/document
https://hal.sorbonne-universite.fr/hal-03152405/document
Autor:
Heather D. Durham, Raphael Munoz-Ruiz, Doris Lou Demy, Edor Kabashi, María Letizia Campanari, Benoit J. Gentil
Neurofilaments (NFs), a major cytoskeletal component of motor neurons, play a key role in their differentiation, establishment and maintenance of their morphology and mechanical strength. Thede novoassembly of these neuronal intermediate filaments re
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4bc53ba634dedddbc18e98f9f6575159
https://doi.org/10.1101/2020.04.27.064063
https://doi.org/10.1101/2020.04.27.064063
Autor:
Marie Ménade, Kalle Gehring, Benoit J. Gentil, Roxanne Larivière, Sandra Minotti, Gia-Thanh Lai, Bernard Brais, J. Paul Chapple, Heather D. Durham
Publikováno v:
The FASEB Journal. 33:2982-2994
Loss of sacsin, a large 520 kDa multidomain protein, causes autosomal recessive spastic ataxia of the Charlevoix-Saguenay, one of the most common childhood-onset recessive ataxias. A prominent feature is abnormal bundling of neurofilaments in many ne
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::55cf3344709a8652beb15888387a9db6
https://doi.org/10.1096/fj.201801556r
https://doi.org/10.1096/fj.201801556r
Autor:
Andreas Hermann, Arun Pal, Heather D. Durham, Kyle St. Louis, Sandra Minotti, Mario Fernandez, Rachel Kuta, Josephine Nalbantoglu, Michael Tibshirani, Nancy Larochelle, Benoit J. Gentil
Publikováno v:
Cell Stress & Chaperones
Cell stress & chaperones 25(1), 173-191 (2020). doi:10.1007/s12192-019-01064-1
Cell stress & chaperones 25(1), 173-191 (2020). doi:10.1007/s12192-019-01064-1
Upregulation of heat shock proteins (HSPs) is an approach to treatment of neurodegenerative disorders with impaired proteostasis. Many neurons, including motor neurons affected in amyotrophic lateral sclerosis (ALS), are relatively resistant to stres
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::b788104ea63c53e126e0be78e5ed78d4
https://pubmed.ncbi.nlm.nih.gov/31900865
https://pubmed.ncbi.nlm.nih.gov/31900865
Publikováno v:
Cell and Tissue Research. 360:609-620
Neurons are extremely polarised cells in which the cytoskeleton, composed of microtubules, microfilaments and neurofilaments, plays a crucial role in maintaining structure and function. Neurofilaments, the 10-nm intermediate filaments of neurons, pro
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::065fd18acf0bf062a22b610c2f387494
https://doi.org/10.1007/s00441-014-2082-7
https://doi.org/10.1007/s00441-014-2082-7