1.Peroxisomal disorders.
Hanyang Medical Reviews 2005;25(3):42-48
Peroxisomes are subcellular organells catalyzing a number of important functions in cellular metabolism. Their functions are mostly related to lipid metabolism. Genetic disorders of peroxisomes are divided into 2 categories: peroxisomal biogenesis disorders and single peroxisomal enzyme deficiencies. This paper describes an overview of the peroxisomal disorders, including metabolic basis, and clinical and laboratory findings.
Lipid Metabolism
;
Metabolism
;
Peroxisomal Disorders*
;
Peroxisomes
;
Organelle Biogenesis
2.Mitochondrial Homeostasis Molecules: Regulation by a Trio of Recessive Parkinson's Disease Genes.
Ji Young HAN ; Ji Soo KIM ; Jin H SON
Experimental Neurobiology 2014;23(4):345-351
Mitochondria are small organelles that produce the majority of cellular energy as ATP. Mitochondrial dysfunction has been implicated in the pathogenesis of Parkinson's disease (PD), and rare familial forms of PD provide valuable insight into the pathogenic mechanism underlying mitochondrial impairment, even though the majority of PD cases are sporadic. The regulation of mitochondria is crucial for the maintenance of energy-demanding neuronal functions in the brain. Mitochondrial biogenesis and mitophagic degradation are the major regulatory pathways that preserve optimal mitochondrial content, structure and function. In this mini-review, we provide an overview of the mitochondrial quality control mechanisms, emphasizing regulatory molecules in mitophagy and biogenesis that specifically interact with the protein products of three major recessive familial PD genes, PINK1, Parkin and DJ-1.
Adenosine Triphosphate
;
Brain
;
Homeostasis*
;
Mitochondria
;
Mitochondrial Degradation
;
Neurons
;
Organelles
;
Parkinson Disease*
;
Quality Control
;
Organelle Biogenesis
3.Rescue of Heart Failure by Mitochondrial Recovery.
Jubert MARQUEZ ; Sung Ryul LEE ; Nari KIM ; Jin HAN
International Neurourology Journal 2016;20(1):5-12
Heart failure (HF) is a multifactorial disease brought about by numerous, and oftentimes complex, etiological mechanisms. Although well studied, HF continues to affect millions of people worldwide and current treatments can only prevent further progression of HF. Mitochondria undoubtedly play an important role in the progression of HF, and numerous studies have highlighted mitochondrial components that contribute to HF. This review presents an overview of the role of mitochondrial biogenesis, mitochondrial oxidative stress, and mitochondrial permeability transition pore in HF, discusses ongoing studies that attempt to address the disease through mitochondrial targeting, and provides an insight on how these studies can affect future research on HF treatment.
Heart Failure*
;
Heart*
;
Mitochondria
;
Organelle Biogenesis
;
Oxidative Stress
;
Permeability
;
Protein Processing, Post-Translational
4.Small Non-coding Transfer RNA-Derived RNA Fragments (tRFs): Their Biogenesis, Function and Implication in Human Diseases.
Yu FU ; Inhan LEE ; Yong Sun LEE ; Xiaoyong BAO
Genomics & Informatics 2015;13(4):94-101
tRNA-derived RNA fragments (tRFs) are an emerging class of non-coding RNAs (ncRNAs). A growing number of reports have shown that tRFs are not random degradation products but are functional ncRNAs made of specific tRNA cleavage. They play regulatory roles in several biological contexts such as cancer, innate immunity, stress responses, and neurological disorders. In this review, we summarize the biogenesis and functions of tRFs.
Organelle Biogenesis*
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Humans*
;
Immunity, Innate
;
Nervous System Diseases
;
Neurodegenerative Diseases
;
RNA*
;
RNA, Transfer
;
RNA, Untranslated
5.AMPK and Exercise: Glucose Uptake and Insulin Sensitivity.
Diabetes & Metabolism Journal 2013;37(1):1-21
AMPK is an evolutionary conserved sensor of cellular energy status that is activated during exercise. Pharmacological activation of AMPK promotes glucose uptake, fatty acid oxidation, mitochondrial biogenesis, and insulin sensitivity; processes that are reduced in obesity and contribute to the development of insulin resistance. AMPK deficient mouse models have been used to provide direct genetic evidence either supporting or refuting a role for AMPK in regulating these processes. Exercise promotes glucose uptake by an insulin dependent mechanism involving AMPK. Exercise is important for improving insulin sensitivity; however, it is not known if AMPK is required for these improvements. Understanding how these metabolic processes are regulated is important for the development of new strategies that target obesity-induced insulin resistance. This review will discuss the involvement of AMPK in regulating skeletal muscle metabolism (glucose uptake, glycogen synthesis, and insulin sensitivity).
Animals
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Glucose
;
Glycogen
;
Insulin
;
Insulin Resistance
;
Mice
;
Organelle Biogenesis
;
Muscle, Skeletal
;
Obesity
6.Insulin Resistance and Intracellular Thyroid Hormone Dysfunction.
Journal of Korean Society of Pediatric Endocrinology 2009;14(2):94-99
Insulin resistance and diabetes combine to impair mitochondrial oxidative metabolism and cause lipid accumulation in non-adipose tissues such as skeletal muscles and the liver. The thyroid hormone stimulates thermogenesis, mitochondrial biogenesis, and various metabolisms, including gluconeogenesis and fatty-acid oxidation. Therefore, altered thyroid hormone action may induce the mitochondrial phenotype associated with insulin resistance. This review focuses on the correlation between thyroid hormone function and diabetes and the possible mechanisms associated with intracellular thyroid hormone dysfunction due to impaired metabolism.
Gluconeogenesis
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Insulin
;
Insulin Resistance
;
Liver
;
Organelle Biogenesis
;
Muscle, Skeletal
;
Phenotype
;
Thermogenesis
;
Thyroid Gland
7.Skeletal Muscle Glycogen Breakdown According to Duration of Endurance Training.
The Korean Journal of Sports Medicine 2016;34(2):101-106
Endurance exercise training such as marathon can increase the ability of exercise performance. Muscle glycogen is associated with an exercise performance, because glycogen depletion is primary causes of muscle fatigue. This review summarizes the glycogen saving effect according to duration of endurance exercise training. Long-term endurance exercise-induced mitochondrial biogenesis contributes to glycogen saving effect that is reduced glycogen breakdown and lactate accumulation. Glycogen sparing is due to a smaller decrease in adenosine triphosphate and phosphocreatine and a smaller increase in inorganic phosphate in the working muscles. It takes required endurance exercise training for about 4 weeks or more. Single bout or short-term endurance exercise is not sufficient to bring an increase in functional mitochondria. But peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) increases rapidly after single bout of endurance exercise. PGC-1α downregulates glycogenolytic and glycolytic enzymes to reduce muscle glycogen breakdown and lactic acid accumulation after short-term endurance exercise.
Adenosine Triphosphate
;
Glycogen*
;
Glycogenolysis
;
Lactic Acid
;
Mitochondria
;
Muscle Fatigue
;
Muscle, Skeletal*
;
Muscles
;
Organelle Biogenesis
;
Peroxisomes
;
Phosphocreatine
8.Role of LAMMER Kinase in Cell Wall Biogenesis during Vegetative Growth of Aspergillus nidulans.
Yu Kyung CHOI ; Eun Hye KANG ; Hee Moon PARK
Mycobiology 2014;42(4):422-426
Depending on the acquisition of developmental competence, the expression of genes for beta-1,3-glucan synthase and chitin synthase was affected in different ways by Aspergillus nidulans LAMMER kinase. LAMMER kinase deletion, DeltalkhA, led to decrease in beta-1,3-glucan, but increase in chitin content. The DeltalkhA strain was also resistant to nikkomycin Z.
Aspergillus nidulans*
;
Organelle Biogenesis*
;
Cell Wall*
;
Chitin
;
Chitin Synthase
;
Mental Competency
;
Phosphotransferases*
9.Roles of Outer Membrane Vesicles (OMVs) in Bacterial Virulence.
Journal of Bacteriology and Virology 2015;45(1):1-10
Outer membrane vesicles (OMVs) are ubiquitous membranous structures in all Gram-negative bacteria, including pathogens and non-pathogens. Gram-positive bacteria also release membrane-derived vesicles (MV). Originating from the cell envelope, OMVs are enriched with bacterial antigen molecules that conduct multiple functions as decoys to manipulate the host immune system. Besides, OMVs and their components play diverse roles in nutrient acquisition, biofilm formation, and resistance to antibiotics. Despite the diverse benefits ascribed to OMVs, many questions remain unanswered with regard to OMV biogenesis and cargo selectivity. In this report, we review the advantages of vesiculation in the context of all bacteria and then focus on additional benefits acquired by OMVs in pathogenic bacteria.
Anti-Bacterial Agents
;
Bacteria
;
Biofilms
;
Gram-Negative Bacteria
;
Gram-Positive Bacteria
;
Immune System
;
Membranes*
;
Virulence*
;
Organelle Biogenesis
10.Promise of Neurorestoration and Mitochondrial Biogenesis in Parkinson's Disease with Multi Target Drugs: An Alternative to Stem Cell Therapy.
Moussa B H YOUDIM ; Young J OH
Experimental Neurobiology 2013;22(3):167-172
There is an unmet need in progressive neurodegenerative diseases such as Parkinson's and Alzheimer's diseases. The present therapeutics for these diseases at best is symptomatic and is not able to delay disease or possess disease modifying activity. Thus an approach to drug design should be made to slow or halt progressive course of a neurological disorder by interfering with a disease-specific pathogenetic process. This would entail the ability of the drug to protect neurons by blocking the common pathway for neuronal injury and cell death and the ability to promote regeneration of neurons and restoration of neuronal function. We have now developed a number of multi target drugs which possess neuroprotective, and neurorestorative activity as well as being able to active PGC-1alpha (peroxisome proliferator-activated receptor gamma coactivator-1alpha), SIRT1 (NAD-dependent deacetylase protein) and NTF (mitochondrial transcription factor) that are intimately associated with mitochondrial biogenesis.
Cell Death
;
Drug Design
;
Nervous System Diseases
;
Neurodegenerative Diseases
;
Neurons
;
Parkinson Disease
;
Regeneration
;
Stem Cells
;
Organelle Biogenesis