Targeting of Monomer/Misfolded SOD1 as a Therapeutic Strategy for
... SOD1 transgenic mice. The specific region(s) of the SOD1 protein to which these immunization approaches target are, however, unknown. In contrast, we have previously shown, using a specific antibody [SOD1 exposed dimer interface (SEDI) antibody], that the dimer interface of SOD1 is abnormally expose ...
... SOD1 transgenic mice. The specific region(s) of the SOD1 protein to which these immunization approaches target are, however, unknown. In contrast, we have previously shown, using a specific antibody [SOD1 exposed dimer interface (SEDI) antibody], that the dimer interface of SOD1 is abnormally expose ...
Full Text
... indistinguishable intracellular distribution in PC12 cells. Cultured primary motor neurons expressing GFP or GFP-SOD1 showed identical patterns of cytoplasmic expression and of movement within the axon. However, GFP tagging of SOD1 was found to alter some of the intrinsic properties of SOD1, includi ...
... indistinguishable intracellular distribution in PC12 cells. Cultured primary motor neurons expressing GFP or GFP-SOD1 showed identical patterns of cytoplasmic expression and of movement within the axon. However, GFP tagging of SOD1 was found to alter some of the intrinsic properties of SOD1, includi ...
Loss of TDP-43 causes age-dependent progressive motor neuron
... Laboratory for Motor Neuron Disease, RIKEN Brain Science Institute, Wako, Saitama 351-0198, Japan CREST, Japan Science and Technology Agency, 4-1-8, Honcho, Kawaguchi, Saitama 332-0012, Japan Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto 606-8507, Japan Department of P ...
... Laboratory for Motor Neuron Disease, RIKEN Brain Science Institute, Wako, Saitama 351-0198, Japan CREST, Japan Science and Technology Agency, 4-1-8, Honcho, Kawaguchi, Saitama 332-0012, Japan Department of Neurology, Kyoto University Graduate School of Medicine, Kyoto 606-8507, Japan Department of P ...
Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and
... lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. Although the identity of the ubiquitinated protein specific to either disorder was unknown, we showed that TDP-43 is the major disease protein in both disorders. Pathologic TDP-43 was hyperphosphorylated, ubiqui ...
... lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. Although the identity of the ubiquitinated protein specific to either disorder was unknown, we showed that TDP-43 is the major disease protein in both disorders. Pathologic TDP-43 was hyperphosphorylated, ubiqui ...
A compensatory subpopulation of motor neurons in a mouse model
... imaged individual neuromuscular junctions in the sternomastoid muscle at multiple time points (see Materials and Methods) to obtain a cellular description of the pathological course of the disease. In wild-type animals, junctions are very stable, showing few alterations in presynaptic terminal branc ...
... imaged individual neuromuscular junctions in the sternomastoid muscle at multiple time points (see Materials and Methods) to obtain a cellular description of the pathological course of the disease. In wild-type animals, junctions are very stable, showing few alterations in presynaptic terminal branc ...
Age-Related Uptake of Heavy Metals in Human Spinal Interneurons
... central nervous system in diseases such as ALS/MND, but judging if a patient died with, or from, a toxicant is a challenge. The toxicant could have entered the nervous system years before the disease become clinically apparent and could no longer be detectable at the time of post mortem examination. ...
... central nervous system in diseases such as ALS/MND, but judging if a patient died with, or from, a toxicant is a challenge. The toxicant could have entered the nervous system years before the disease become clinically apparent and could no longer be detectable at the time of post mortem examination. ...
Trigeminal pathways handout
... 3. Diagram the corneal reflex: the afferent and efferent limbs as well as nuclei involved in the brainstem. 4. If a person does not blink, how would you determine if the problem were in the sensory (afferent) limb, motor (efferent) limb, or brainstem interconnections for the corneal reflex? 5. Expla ...
... 3. Diagram the corneal reflex: the afferent and efferent limbs as well as nuclei involved in the brainstem. 4. If a person does not blink, how would you determine if the problem were in the sensory (afferent) limb, motor (efferent) limb, or brainstem interconnections for the corneal reflex? 5. Expla ...
Degeneration and impaired regeneration of gray matter
... ROSA26-EYFP; SOD1 (G93A) and littermate control mice received 4HT (2 mg) at P35 and were analyzed at P40, P50, P90 and P120 (or end stage for ALS mice). (b) Schematic showing the rationale and possible outcomes of oligodendrocyte fate analysis. (c,d) Plot of the number of EYFP+ oligodendrocytes (CC1 ...
... ROSA26-EYFP; SOD1 (G93A) and littermate control mice received 4HT (2 mg) at P35 and were analyzed at P40, P50, P90 and P120 (or end stage for ALS mice). (b) Schematic showing the rationale and possible outcomes of oligodendrocyte fate analysis. (c,d) Plot of the number of EYFP+ oligodendrocytes (CC1 ...
Study of Trace and Minor ... ALS (Amyotrophic Lateral Sclerosis) Patients by J.
... dementia, muscular weakness, and atrophy. While not as common as Alzheimer's disease, ...
... dementia, muscular weakness, and atrophy. While not as common as Alzheimer's disease, ...
ASCENDING WHITE MATTER TRACTS
... 90% project to IN of lamina VII and VIII; 10% project to MN of lamina IX of distal muscles Anterior CS: anterior funiculus; only in cervical and upper T levels; innervates axial neck/body muscles; ...
... 90% project to IN of lamina VII and VIII; 10% project to MN of lamina IX of distal muscles Anterior CS: anterior funiculus; only in cervical and upper T levels; innervates axial neck/body muscles; ...
Frontal lobe dysfunction in amyotrophic lateral sclerosis
... between the two patient groups in the separate bulbar and spinal function disability scores, although a non-significant trend emerged for the comparison of total disability scores [F(l,lO) =4.39, P = 0.06] which was significant using a 95% confidence interval (0.2, 5.4), with the ALSi group tending ...
... between the two patient groups in the separate bulbar and spinal function disability scores, although a non-significant trend emerged for the comparison of total disability scores [F(l,lO) =4.39, P = 0.06] which was significant using a 95% confidence interval (0.2, 5.4), with the ALSi group tending ...
Deleterious effects of amyloid beta peptide in the neuromuscular
... neuromotor system. It has been shown that Amyloid-beta (Aβ) levels are elevated in spinal cords of late-stage superoxide dismutase 1 (SOD1) G93A mice (model of familial amyotrophic lateral sclerosis [ALS]) and that Aβ peptide(s) were localized predominantly within affected motor neurons (MN) and sur ...
... neuromotor system. It has been shown that Amyloid-beta (Aβ) levels are elevated in spinal cords of late-stage superoxide dismutase 1 (SOD1) G93A mice (model of familial amyotrophic lateral sclerosis [ALS]) and that Aβ peptide(s) were localized predominantly within affected motor neurons (MN) and sur ...
Lecture 3 Figure 1
... Neurotransmitters: Corticotectal projections, especially those from the visual cortex, utilize glutamate (⫹). This substance is also present in most corticoreticular fibers. Some neurons of the gigantocellular reticular nucleus that send their axons to the spinal cord, as reticulospinal projections, ...
... Neurotransmitters: Corticotectal projections, especially those from the visual cortex, utilize glutamate (⫹). This substance is also present in most corticoreticular fibers. Some neurons of the gigantocellular reticular nucleus that send their axons to the spinal cord, as reticulospinal projections, ...
full program with abstracts
... A new method to measure proteostasis capacity: a potential biosensor for pre-onset neurodegenerative disease A/Prof Danny Hatters, University of Melbourne Protein-folding homeostasis (proteostasis) in humans is controlled by a network of about 800 proteins. While proteostasis can dynamically respond ...
... A new method to measure proteostasis capacity: a potential biosensor for pre-onset neurodegenerative disease A/Prof Danny Hatters, University of Melbourne Protein-folding homeostasis (proteostasis) in humans is controlled by a network of about 800 proteins. While proteostasis can dynamically respond ...
Tese final so frentes - Repositório da Universidade de Lisboa
... It was in the latter half of the 19th century that the initial steps towards the unraveling of one of the most common motor neuron diseases (MND) were accomplished. Using clinical cases and autopsy material, a technique known as “anatomo-clinical method”, the famous French neurobiologist and physici ...
... It was in the latter half of the 19th century that the initial steps towards the unraveling of one of the most common motor neuron diseases (MND) were accomplished. Using clinical cases and autopsy material, a technique known as “anatomo-clinical method”, the famous French neurobiologist and physici ...
Non-Cell-Autonomous Effect of Human SOD1G37R
... hESC-derived rosettes expressed motor neuron progenitor markers such as Pax6, Nestin, Olig2, and Islet1 after 2–3 weeks of differentiation (Figures 1A–1D). After 4 weeks under differentiation conditions, the cells started to express panneuronal markers such as TuJ1, and after 6–8 weeks, the cells ex ...
... hESC-derived rosettes expressed motor neuron progenitor markers such as Pax6, Nestin, Olig2, and Islet1 after 2–3 weeks of differentiation (Figures 1A–1D). After 4 weeks under differentiation conditions, the cells started to express panneuronal markers such as TuJ1, and after 6–8 weeks, the cells ex ...
TESE
... may occur within the same family, often with an autosomaldominant pattern of inheritance. This familial association is not well explained by the currently recognized genetic defects; GRN mutations are not associated with significant motor neuron deficits, while patients carrying mutations in SOD1, T ...
... may occur within the same family, often with an autosomaldominant pattern of inheritance. This familial association is not well explained by the currently recognized genetic defects; GRN mutations are not associated with significant motor neuron deficits, while patients carrying mutations in SOD1, T ...
Morphological Studies of Wobbler Mouse Dorsal Root Ganglia
... (αMN) have received the most attention. With regard to pathological cellular processes, particularly those of impaired axonal transport, neuronal tissues in general should be examined. Dorsal root ganglia (DRG) cells are equipped with extremely long axons. Thus, we expected them to be an excellent t ...
... (αMN) have received the most attention. With regard to pathological cellular processes, particularly those of impaired axonal transport, neuronal tissues in general should be examined. Dorsal root ganglia (DRG) cells are equipped with extremely long axons. Thus, we expected them to be an excellent t ...
Abnormal Electric Activity Insertional Activity --Normal is 100
... --seen in: myasthenia gravis, botulism, early reinnervation after nerve damage, late stage neurogenic atrophy, muscular dystrophies, periodic paralysis, polymyositis, toxic myopathies, congenital myopathies Long-Duration MUPs --commonly high amplitude with poor recruitment --occur when there is an i ...
... --seen in: myasthenia gravis, botulism, early reinnervation after nerve damage, late stage neurogenic atrophy, muscular dystrophies, periodic paralysis, polymyositis, toxic myopathies, congenital myopathies Long-Duration MUPs --commonly high amplitude with poor recruitment --occur when there is an i ...
PDF here
... The spinal cord was fixed in situ with 4% buffered paraformaldehyde for 48 h. The entire lumbar enlargement was then dissected, embedded in paraffin wax, and exhaustively cross-sectioned at 8 AM, six sections to a slide (123 F 12 slides or 5.90 F 0.58 mm of tissue per specimen). Tissue was deparaffi ...
... The spinal cord was fixed in situ with 4% buffered paraformaldehyde for 48 h. The entire lumbar enlargement was then dissected, embedded in paraffin wax, and exhaustively cross-sectioned at 8 AM, six sections to a slide (123 F 12 slides or 5.90 F 0.58 mm of tissue per specimen). Tissue was deparaffi ...
![[10] P. Paul, J de Belleroche, The role of D-amino acids in](http://s1.studyres.com/store/data/022394228_1-c70b74890df8cd7f8a841431fb6562f6-300x300.png)
[10] P. Paul, J de Belleroche, The role of D-amino acids in
... respectively. These are nuclear proteins but they mislocalise to the cytoplasm in disease and ...
... respectively. These are nuclear proteins but they mislocalise to the cytoplasm in disease and ...
Neuromuscular Emergencies - S Derghazarian 07 28 10
... National Institute of Neurological Disorders and Stroke (NINDS) criteria are based on expert consensus. Required features include: – Progressive weakness of more than one limb, ranging from minimal weakness of the legs to total paralysis of all four limbs, the trunk, bulbar and facial muscles, and e ...
... National Institute of Neurological Disorders and Stroke (NINDS) criteria are based on expert consensus. Required features include: – Progressive weakness of more than one limb, ranging from minimal weakness of the legs to total paralysis of all four limbs, the trunk, bulbar and facial muscles, and e ...
Mitochondrial dysfunction in neurodegenerative disorders
... caused by a progressive degeneration of the anterior horn cells of the spinal cord and cortical motor neurons. The primary cause of the neuronal cell death in ALS so far remains unclear. Some early concepts relate the neurodegenerative process to glutamate-induced excitotoxicity [29]. There is compe ...
... caused by a progressive degeneration of the anterior horn cells of the spinal cord and cortical motor neurons. The primary cause of the neuronal cell death in ALS so far remains unclear. Some early concepts relate the neurodegenerative process to glutamate-induced excitotoxicity [29]. There is compe ...
View CV as a PDF - Cedars
... My current research focus combines neuroscience and regenerative medicine in order to understand mechanisms of disease and neurological disorders such as ALS and traumatic brain injury with the goal of developing therapeutic treatments. ...
... My current research focus combines neuroscience and regenerative medicine in order to understand mechanisms of disease and neurological disorders such as ALS and traumatic brain injury with the goal of developing therapeutic treatments. ...
Stephen Hawking
... spinal cord. Motor neurons are nerve cells that control your movement. The upper and lower motor neurons are an important part of the body’s neuromuscular system. Over time, ALS causes these motor neurons to ...
... spinal cord. Motor neurons are nerve cells that control your movement. The upper and lower motor neurons are an important part of the body’s neuromuscular system. Over time, ALS causes these motor neurons to ...
Amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease and Charcot disease, is a specific disorder that involves the death of neurons. In the United Kingdom the term motor neurone disease (MND) is commonly used, while others use that term for a group of five conditions of which ALS is the most common. ALS is characterized by stiff muscles, muscle twitching, and gradually worsening weakness due to muscle wasting. This results in difficulty speaking, swallowing, and eventually breathing.The cause is not known in 90% to 95% of cases. About 5–10% of cases are inherited from a person's parents. About half of these genetic cases are due to one of two specific genes. It results in the death of the neurons that control voluntary muscles. The diagnosis is based on a person's signs and symptoms with testing done to rule out other potential causes.There is no cure for ALS. A medication called riluzole may extend life expectancy by about two to three months. Non-invasive ventilation may result in both improved quality and length of life. The disease usually starts around the age of 60 and in inherited cases around the age of 50. The average survival from onset to death is three to four years. About 10% survive longer than 10 years. Most die from respiratory failure. In much of the world, rates of ALS are unknown. In Europe and the United States, the disease affects about 2 people per 100,000 per year.Descriptions of the disease date back to at least 1824 by Charles Bell. In 1869, the connection between the symptoms and the underlying neurological problems were first described by Jean-Martin Charcot, who in 1874 began using the term amyotrophic lateral sclerosis. It became well known in the United States in the 20th century when it affected the baseball player Lou Gehrig, and later when Stephen Hawking gained fame for his scientific achievements. In 2014 videos of the ice bucket challenge went viral on the internet and increased public awareness.