Downregulation of VAPB expression in motor neurons derived from induced pluripotent stem cells of ALS8 patients
M Mitne-Neto, M Machado-Costa… - Human molecular …, 2011 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) is an incurable neuromuscular disease that leads to a
profound loss of life quality and premature death. Around 10% of the cases are inherited and …
profound loss of life quality and premature death. Around 10% of the cases are inherited and …
The link between VAPB loss of function and amyotrophic lateral sclerosis
N Borgese, N Iacomino, SF Colombo, F Navone - Cells, 2021 - mdpi.com
The VAP proteins are integral adaptor proteins of the endoplasmic reticulum (ER)
membrane that recruit a myriad of interacting partners to the ER surface. Through these …
membrane that recruit a myriad of interacting partners to the ER surface. Through these …
A Drosophila Model of ALS: Human ALS-Associated Mutation in VAP33A Suggests a Dominant Negative Mechanism
A Ratnaparkhi, GM Lawless, FE Schweizer… - PloS one, 2008 - journals.plos.org
ALS8 is caused by a dominant mutation in an evolutionarily conserved protein, VAPB
(vesicle-associated membrane protein (VAMP)-associated membrane protein B)/ALS8). We …
(vesicle-associated membrane protein (VAMP)-associated membrane protein B)/ALS8). We …
Investigating the contribution of VAPB/ALS8 loss of function in amyotrophic lateral sclerosis
The mutations P56S and T46I in the gene encoding vesicle-associated membrane protein-
associated protein B/C (VAPB) cause ALS8, a familial form of amyotrophic lateral sclerosis …
associated protein B/C (VAPB) cause ALS8, a familial form of amyotrophic lateral sclerosis …
Induced pluripotent stem cells and their applications in amyotrophic lateral sclerosis
H Du, Z Huo, Y Chen, Z Zhao, F Meng, X Wang, S Liu… - Cells, 2023 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that results
in the loss of motor function in the central nervous system (CNS) and ultimately death. The …
in the loss of motor function in the central nervous system (CNS) and ultimately death. The …
A comprehensive library of familial human amyotrophic lateral sclerosis induced pluripotent stem cells
Y Li, U Balasubramanian, D Cohen, PW Zhang… - PloS one, 2015 - journals.plos.org
Amyotrophic lateral sclerosis is a progressive disease characterized by the loss of upper
and lower motor neurons, leading to paralysis of voluntary muscles. About 10% of all ALS …
and lower motor neurons, leading to paralysis of voluntary muscles. About 10% of all ALS …
Induced pluripotent stem cells from ALS patients for disease modeling
JP Richard, NJ Maragakis - Brain research, 2015 - Elsevier
The ability to reprogram adult somatic cells into pluripotent stem cells that can differentiate
into all three germ layers of the developing human has fundamentally changed the …
into all three germ layers of the developing human has fundamentally changed the …
Drug screening for ALS using patient-specific induced pluripotent stem cells
N Egawa, S Kitaoka, K Tsukita, M Naitoh… - Science translational …, 2012 - science.org
Amyotrophic lateral sclerosis (ALS) is a late-onset, fatal disorder in which the motor neurons
degenerate. The discovery of new drugs for treating ALS has been hampered by a lack of …
degenerate. The discovery of new drugs for treating ALS has been hampered by a lack of …
A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells
MF Burkhardt, FJ Martinez, S Wright, C Ramos… - Molecular and Cellular …, 2013 - Elsevier
Abstract Development of therapeutics for genetically complex neurodegenerative diseases
such as sporadic amyotrophic lateral sclerosis (ALS) has largely been hampered by lack of …
such as sporadic amyotrophic lateral sclerosis (ALS) has largely been hampered by lack of …
Modeling ALS with motor neurons derived from human induced pluripotent stem cells
Directing the differentiation of induced pluripotent stem cells into motor neurons has allowed
investigators to develop new models of amyotrophic lateral sclerosis (ALS). However …
investigators to develop new models of amyotrophic lateral sclerosis (ALS). However …