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Articles 1 - 12 of 12
Full-Text Articles in Biochemistry
Mitochondrial Roles In Developmentally Programmed Heart Disease, Eli John Louwagie
Mitochondrial Roles In Developmentally Programmed Heart Disease, Eli John Louwagie
Dissertations and Theses
Offspring of diabetic and obese mothers (ODOM) have greater risks of heart disease at birth and later in life. However, prevention is hindered because underlying mechanisms are poorly understood. Mounting studies in the Developmental Origins of Health and Disease field suggest that mitochondria play key roles in developmentally programmed heart disease similar to the roles they play in cardiomyopathy in adults with diabetes and obesity. However, whether mitochondria are responsible for the short[1]and long-term cardiac disease seen in ODOM remains unknown. Here, we sought to delineate the roles of mitochondria in the hearts of ODOM, determine whether mitochondria are playing …
Therapies For Mitochondrial Disorders, Kayli Sousa Smyth, Anne Mulvihill
Therapies For Mitochondrial Disorders, Kayli Sousa Smyth, Anne Mulvihill
SURE Journal: Science Undergraduate Research Experience Journal
Mitochondria are cytoplasmic, double-membrane organelles that synthesise adenosine triphosphate (ATP). Mitochondria contain their own genome, mitochondrial DNA (mtDNA), which is maternally inherited from the oocyte. Mitochondrial proteins are encoded by either nuclear DNA (nDNA) or mtDNA, and both code for proteins forming the mitochondrial oxidative phosphorylation (OXPHOS) complexes of the respiratory chain. These complexes form a chain that allows the passage of electrons down the electron transport chain (ETC) through a proton motive force, creating ATP from adenosine diphosphate (ADP). This study aims to explore current and prospective therapies for mitochondrial disorders (MTDS). MTDS are clinical syndromes coupled with abnormalities …
Ankyrin Dependent Mitochondrial Function And Bioenergetics In The Heart, Janani Subramaniam, Janani Subramaniam
Ankyrin Dependent Mitochondrial Function And Bioenergetics In The Heart, Janani Subramaniam, Janani Subramaniam
Dissertations & Theses (Open Access)
ANK2 mutations in patients are associated with numerous arrhythmias, cardiomyopathies, and other heart defects. In the heart, AnkB, the protein encoded by ANK2, clusters relevant ion channels and cell adhesion molecules in several important domains; however, its role at Mitochondria Associated ER/SR Membranes (MAMs) has yet to be investigated. MAMs are crucial to mitochondrial function and metabolism and are signaling hubs implicated in various cardiac pathologies. Among several functions, these sites mediate the direct transfer of calcium from the ER/SR to the mitochondria to modulate ATP synthesis. Given that mitochondrial function and energy production are paramount to cardiovascular heath, …
Evaluating Bioenergetics And Mitochondrial Dynamics In Patient Fibroblasts With Pathogenic Mitochondrial Dna Mutations Causing Leigh Syndrome, Ajibola Bakare
Evaluating Bioenergetics And Mitochondrial Dynamics In Patient Fibroblasts With Pathogenic Mitochondrial Dna Mutations Causing Leigh Syndrome, Ajibola Bakare
Graduate Theses and Dissertations
Leigh syndrome (LS) is a rare fatal mitochondrial disorder of infants caused by pathogenic mutations in the nuclear (nDNA) or mitochondrial DNA (mtDNA) leading to mitochondrial dysfunction. The extent to which pathogenic mtDNA variants regulate disease severity in LS is not well understood. The heterogeneous nature of this disorder, based in part by complex mitochondrial genetics, and the nuclear and mitochondrial cross-talk has made it particularly challenging to investigate and develop therapies for treating LS . While the prognosis is poor, several studies are underway to understand the pathophysiology of LS. This dissertation provides a comprehensive structural and functional analysis …
Quantification Of Mitochondrial Zinc Homeostasis And Analysis Of Zinc And Polyamine-Mediated Axonal Trafficking, Dylan Hunt Fudge
Quantification Of Mitochondrial Zinc Homeostasis And Analysis Of Zinc And Polyamine-Mediated Axonal Trafficking, Dylan Hunt Fudge
Electronic Theses and Dissertations
The zinc ion is an important emerging signaling molecule for biological processes. In this work we engineered improved zinc sensors based on our previously developed fluorescent sensor GZnP1 to provide sensors with a higher fluorescent readout, faster kinetics, and a superior sensitivity to zinc. We utilized these zinc sensors and further developed the sensors to answer questions pertaining to biological zinc. We showed the labile zinc concentration in the mitochondrial matrix was less than 1 pM while the labile zinc concentration in the cytosol and mitochondrial IMS were comparable (~100 pM) across four different cell lines. Using these zinc sensors …
Seasonal Variation In Mitochondrial Bioenergetics Of The Bluegill Sunfish, Lepomis Macrochirus, From A Shallow Midwest River, Derick Isaac Lamptey
Seasonal Variation In Mitochondrial Bioenergetics Of The Bluegill Sunfish, Lepomis Macrochirus, From A Shallow Midwest River, Derick Isaac Lamptey
Masters Theses
As average global temperature increase, the frequency and magnitude of extreme temperatures in shallow aquatic ecosystems are more ubiquitous. In order to understand how these changing thermal regimes affect aquatic ectotherms, it is essential to develop studies evaluating the response of ectotherms to seasonal fluctuating thermal regimes. Previous studies on fluctuating temperature regimes have reported an increased physiological stress leading to morphological, behavioral and biochemical adaptations. From the latter, the adaptive capacity and seasonal performance associated with optimal function of the oxidative phosphorylation system (OXPHOS) are key for species persistence. However, studies on this matter are scarce. This study explores …
Deletion Of Mgr2p Affects The Gating Behavior Of The Tim23 Complex, Oygul Mirzalieva, Shinhye Jeon, Kevin Damri, Ruth Hartke, Layla Drwesh, Keren Demishtein-Zohary, Abdussalam Azem, Cory D. Dunn, Pablo M. Peixoto
Deletion Of Mgr2p Affects The Gating Behavior Of The Tim23 Complex, Oygul Mirzalieva, Shinhye Jeon, Kevin Damri, Ruth Hartke, Layla Drwesh, Keren Demishtein-Zohary, Abdussalam Azem, Cory D. Dunn, Pablo M. Peixoto
Publications and Research
The TIM23 complex is a hub for translocation of preproteins into or across the mitochondrial inner membrane. This dual sorting mechanism is currently being investigated, and in yeast appears to be regulated by a recently discovered subunit, the Mgr2 protein. Deletion of Mgr2p has been found to delay protein translocation into the matrix and accumulation in the inner membrane. This result and other findings suggested that Mgr2p controls the lateral release of inner membrane proteins harboring a stop-transfer signal that follows an N-terminal amino acid signal. However, the mechanism of lateral release is unknown. Here, we used patch clamp electrophysiology …
Impact Of San-Mediated Signaling On Glioblastoma And Neuroblastoma Metabolism, Monica Rodriguez Silva
Impact Of San-Mediated Signaling On Glioblastoma And Neuroblastoma Metabolism, Monica Rodriguez Silva
FIU Electronic Theses and Dissertations
Glioblastoma (GBM) is the most common and aggressive type of brain cancer, with an average life expectancy of 15 months. The standard of care for GBM, surgery accompanied by radiation and chemotherapy (temozolomide-TMZ), has not changed in over 10 years illustrating the need for new and efficacious treatments. Therefore, it is imperative to improve our knowledge of GBM physiology to understand the mechanisms driving recurrence and chemoresistance so that more effective therapeutic options can be developed. Mitochondria-cell communication is key to monitor and maintain both mitochondrial and cellular health, and signaling events on the outer mitochondrial membrane (OMM) have emerged …
Axonal Transport And Life Cycle Of Mitochondria In Parkinson's Disease Model, Hyun Sung
Axonal Transport And Life Cycle Of Mitochondria In Parkinson's Disease Model, Hyun Sung
Open Access Dissertations
In neurons, normal distribution and selective removal of mitochondria are essential for preserving compartmentalized cellular function. Parkin, an E3 ubiquitin ligase associated with familial Parkinson’s disease, has been implicated in mitochondrial dynamics and removal. However, it is not clear how Parkin plays a role in mitochondrial turnover in vivo, and whether the mature neurons possess a compartmentalized Parkin-dependent mitochondrial life cycle. Using the live Drosophila nervous system, here, I investigate the involvement of Parkin in mitochondrial dynamics; organelle distribution, morphology and removal. Parkin deficient animals displayed less number of axonal mitochondria without disturbing organelle motility behaviors, morphology and metabolic state. …
Molecular Characterization Of A Isoenzyme Of The Targeting Peptide Degrading Protease, Prep2- Catalysis, Subcellular Localization, Expression And Evolution, S. Bhushan, A. Stahl, S. Nilsson, B. Lefebvre, D. Mcwilliams, S.J. Wright, M. Seki, D.A. Liberles, K. Shinozaki, Barry D. Bruce, M. Boutry, E. Glaser
Molecular Characterization Of A Isoenzyme Of The Targeting Peptide Degrading Protease, Prep2- Catalysis, Subcellular Localization, Expression And Evolution, S. Bhushan, A. Stahl, S. Nilsson, B. Lefebvre, D. Mcwilliams, S.J. Wright, M. Seki, D.A. Liberles, K. Shinozaki, Barry D. Bruce, M. Boutry, E. Glaser
Faculty Publications and Other Works -- Biochemistry, Cellular and Molecular Biology
We have previously identified a zinc metalloprotease involved in the degradation of mitochondrial and chloroplast targeting peptides, the presequence protease (PreP). In the Arabidopsis thaliana genomic database, there are two genes that correspond to the protease, the zinc metalloprotease (AAL90904) and the putative zinc metalloprotease (AAG13049). We have named the corresponding proteins AtPreP1 and AtPreP2, respectively. AtPreP1 and AtPreP2 show significant differences in their targeting peptides and the proteins are predicted to be localized in different compartments. AtPreP1 was shown to degrade both mitochondrial and chloroplast targeting peptides and to be dual targeted to both organelles using an ambiguous targeting …
Molecular Characterization Of A Isoenzyme Of The Targeting Peptide Degrading Protease, Prep2- Catalysis, Subcellular Localization, Expression And Evolution, S. Bhushan, A. Stahl, S. Nilsson, B. Lefebvre, D. Mcwilliams, S.J. Wright, M. Seki, D.A. Liberles, K. Shinozaki, Barry D. Bruce, M. Boutry, E. Glaser
Molecular Characterization Of A Isoenzyme Of The Targeting Peptide Degrading Protease, Prep2- Catalysis, Subcellular Localization, Expression And Evolution, S. Bhushan, A. Stahl, S. Nilsson, B. Lefebvre, D. Mcwilliams, S.J. Wright, M. Seki, D.A. Liberles, K. Shinozaki, Barry D. Bruce, M. Boutry, E. Glaser
Barry D. Bruce
We have previously identified a zinc metalloprotease involved in the degradation of mitochondrial and chloroplast targeting peptides, the presequence protease (PreP). In the Arabidopsis thaliana genomic database, there are two genes that correspond to the protease, the zinc metalloprotease (AAL90904) and the putative zinc metalloprotease (AAG13049). We have named the corresponding proteins AtPreP1 and AtPreP2, respectively. AtPreP1 and AtPreP2 show significant differences in their targeting peptides and the proteins are predicted to be localized in different compartments. AtPreP1 was shown to degrade both mitochondrial and chloroplast targeting peptides and to be dual targeted to both organelles using an ambiguous targeting …
The Effects Of Luteinizing Hormone And Adenosine 3',5'-Cyclic Monophosphate On Phospholipid Metabolism By Luteal Mitochondria, Jim John Sadighian
The Effects Of Luteinizing Hormone And Adenosine 3',5'-Cyclic Monophosphate On Phospholipid Metabolism By Luteal Mitochondria, Jim John Sadighian
Chemistry & Biochemistry Theses & Dissertations
Luteinizing hormone (LH) increases intracellular concentrations of adenosine 3', 5 '-cyclic monophosphate and the phosphoinositides, phosphatidylinositol (PI), PI 4'-phosphate (PIP) and PI 4' .5 1 - bispbosphate (PIP2). It is believed that cAMP and the phosphoinositides act concertedly to regulate mitochondrial conversion of cholesterol to pregnenolone. This study examined the effects of LH and N6 ,O2 -dibutyryl cAMP (dbcAMP) on phospholipids metabolism by luteal mitochondria and the influence of dbcAMP and the phosphoinosi tides on mitochondrial steroid production. Mitochondria were isolated from unincubated and incubated luteal tissue by differential centrifugation. Phospholipids were extracted from the mitochondria …