Open Access. Powered by Scholars. Published by Universities.®
Molecular and Cellular Neuroscience Commons™
Open Access. Powered by Scholars. Published by Universities.®
- Institution
- Keyword
-
- FUS (2)
- Adenosine (1)
- Adenosine a2b (1)
- Adora2b (1)
- Aging (1)
-
- Amyotrophic Lateral Sclerosis (1)
- Autophagy (1)
- Cytoplasmic inclusions (1)
- Hearing loss (1)
- Huntington's Disease (1)
- Lysine acetylation (1)
- Matrix Metalloproteases (1)
- Myelin protein zero (1)
- Neurodegenerative diseases (1)
- Neuroinflammation (1)
- Presbycusis (1)
- Protein Folding (1)
- Protein Quality Control (1)
- R6/2 Mice (1)
- RNA-binding (1)
- Sensorineural hearing loss (1)
- Stress granules (1)
- TDP-43 (1)
- Tissue Inhibitor of Metalloproteases (1)
Articles 1 - 4 of 4
Full-Text Articles in Molecular and Cellular Neuroscience
Dnajc7, A Molecular Chaperone Protein That Modulates Protein Misfolding In Amyotrophic Lateral Sclerosis (Als), Meaghan Kathleen Stoltz
Dnajc7, A Molecular Chaperone Protein That Modulates Protein Misfolding In Amyotrophic Lateral Sclerosis (Als), Meaghan Kathleen Stoltz
Electronic Thesis and Dissertation Repository
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease associated with protein misfolding and dysregulated cellular protein quality control mechanisms. Molecular chaperones, and heat shock proteins (Hsp), are key players in maintaining cellular protein quality control. DNAJC7 is an understudied cytosolic Hsp40 that works together with Hsp70 and Hsp90 to regulate proper protein folding or degradation. Of note, mutations in the gene encoding DNAJC7 were discovered to cause familial ALS. We asked whether ALS-associated mutations in DNAJC7 compromise its function as a chaperone, which may cause the toxic accumulation of misfolded proteins. This study attempts to uncover the functions of DNAJC7 …
Elevated Cochlear Adenosine Causes Hearing Loss Via Adora2b Signaling, Jeanne Manalo
Elevated Cochlear Adenosine Causes Hearing Loss Via Adora2b Signaling, Jeanne Manalo
Dissertations & Theses (Open Access)
Over 538 million people in the world have been diagnosed with hearing loss (HL). Current treatments for the most common type of HL, sensorineural HL, are limited to hearing aids and cochlear implants with no FDA-drugs available. The hearing process demands an abundance of ATP and HL is often attributed to a disruption in this metabolic energy currency. Patients who lack adenosine deaminase (ADA), the enzyme that irreversibly metabolizes adenosine, have high levels of adenosine that yield severe health problems, including HL; however, the pathogenic mechanisms behind HL and adenosine remain elusive. Our lab has found a HL phenotype in …
Modulating Matrix Metalloproteases And Inflammation In Huntington’S Disease, Alejandro Lopez Ramirez
Modulating Matrix Metalloproteases And Inflammation In Huntington’S Disease, Alejandro Lopez Ramirez
Natural Sciences and Mathematics | Biological Sciences Master's Theses
Huntington’s disease (HD) is a rare and incurable autosomal neurodegenerative disease affecting 1-10 in every 100,000 people in the world. There is no cure for HD and treatments available alleviate certain symptoms for short periods of time. Evidence suggests that neuropathology of HD begins with the proteolysis of the mutated Huntingtin (mHTT) protein. A variety of proteases, like the matrix metalloproteases, cleave mHTT creating proteinaceous fragments that are thought to be neurotoxic. As these fragments increase in the brain, the damage to neurons also increases, leading to chronic inflammation due to hyper reactive microglia and astrocytes attempting to minimize and …
Novel Post-Translational Modification And Function Of Fus: The Relevance To Amyotrophic Lateral Sclerosis, Alexandra Arenas
Novel Post-Translational Modification And Function Of Fus: The Relevance To Amyotrophic Lateral Sclerosis, Alexandra Arenas
Theses and Dissertations--Toxicology and Cancer Biology
Amyotrophic Lateral Sclerosis (ALS) is a neurodegenerative disease characterized by the preferential death of motor neurons. Approximately 10% of ALS cases are familial and 90% are sporadic. Fused in Sarcoma (FUS) is a ubiquitously expressed RNA binding protein implicated in familial ALS and frontotemporal dementia (FTD). FUS is ubiquitously expressed in cells and has a variety of functions in the nucleus and cytoplasm. FUS mutations in the nuclear localization sequence (NLS) causes mislocalization of FUS in the cytoplasm, where it can undergo liquid-liquid phase separation and become stress granules or protein inclusions. Although FUS inclusion bodies can be found in …