Browse Items · NEOMED Bibliography Database

Biochemical phenotype and its relationship to treatment in 16 individuals with PCCB c.1606A > G (p.Asn536Asp) variant propionic acidemia.

Propionic acidemia (PA) is caused by inherited deficiency of mitochondrial propionyl-CoA carboxylase (PCC) and results in significant neurodevelopmental and cardiac morbidity. However, relationships among therapeutic intervention, biochemical…

Optical mapping of cryoinjured rat myocardium grafted with mesenchymal stem cells

Costa AR, Panda NC, Yong S, Mayorga ME, Pawlowski GP, Fan K, Penn MS, Laurita KR. Optical mapping of cryoinjured rat myocardium grafted with mesenchymal stem cells. Am J Physiol Heart Circ Physiol 302: H270-H277, 2012. First published October 28,…

Homozygosity for a mutation affecting the catalytic domain of tyrosyl-tRNA synthetase (YARS) causes multisystem disease

Aminoacyl-tRNA synthetases (ARSs) are critical for protein translation. Pathogenic variants of ARSs have been previously associated with peripheral neuropathy and multisystem disease in heterozygotes and homozygotes, respectively. We report seven…

Tags: 2019, Adult, Antonellis Anthony, Baras Aris, Biochemistry & Molecular Biology, Brigatti Karlla W, Carson Vincent J, Catalytic Domain, Child, Demczko Matthew M, Female, Fox Michael D, Furuya Katryn N, gene, Genetic Diseases, Genetic Predisposition to Disease, Genetics & Heredity, Gonzaga-Jauregui Claudia, Griffin Laurie B, Hearing Loss, Heterozygote, Homozygote, Human molecular genetics, Humans, impairment, Inborn, Infant, Jinks Robert N, Kandula Vinay V R, lactic-acidosis, Loss of Function Mutation, Male, Martinez Erick D, mechanisms, Miles Lili, Mutation, myopathy, NEOMED College of Medicine Student, NEOMED Student Publications, Newborn, onset, Overton John D, Pedigree, Phenotype, phenotypic variability, Poskitt Laura, Preschool, Puffenberger Erik G, Radhakrishnan Kadakkal, recessive mutations, Reid Jeffrey G, Sensorineural, September 2019 Update, Severity of Illness Index, Strauss Kevin A, swiss-model, Tyrosine-tRNA Ligase, variant, Wenger Olivia K, Whole Exome Sequencing, Williams Katie B, Yeasts, Yoder Mark A

Ethanol interoceptive cue and sleep-time duration in HAS and LAS selectively bred rats.

The drug discrimination paradigm was used to evaluate the effects of selective breeding for differential sensitivity to the hypnotic effects of ethanol. Tenth generation high alcohol sensitive (HAS) and low alcohol sensitive (LAS) rats were trained…

Tags: *Cues, *Discrimination Learning, *Hypnotics and Sedatives, 1990, Animals, Breeding, Ethanol/*pharmacology, Krimmer E C, Male, Pharmacology, biochemistry, and behavior, Phenotype, Rats, Sleep/*drug effects, Species Specificity, Time Factors

Growth hormone injections improve bone quality in a mouse model of osteogenesis imperfecta.

UNLABELLED: Systemic growth hormone injections increased spine and femur length in a mouse model of OI. Femur BMC, cross-sectional area, and BMD were increased. Smaller gains were produced in vertebral BMC and cross-sectional area. Biomechanical…

Evidence of Oropharyngeal Dysfunction in Feeding in the Rat Rotenone Model of Parkinson's Disease.

Swallowing disorders in Parkinson's disease are not responsive to dopamine depletion therapy and contribute to morbidity. They are poorly understood owing to a lack of adequate models. We present the first evidence of oropharyngeal changes in a…

The role of mitochondrial bioenergetics and reactive oxygen species in coronary collateral growth.

Coronary collateral growth is a process involving coordination between growth factors expressed in response to ischemia and mechanical forces. Underlying this response is proliferation of vascular smooth muscle and endothelial cells, resulting in an…

Impact of type 1 diabetes on cardiac fibroblast activation: enhanced cell cycle progression and reduced myofibroblast content in diabetic myocardium.

Diabetic patients are prone to developing myocardial fibrosis and suffer from decreased wound healing capabilities. The purpose of this study was to determine whether diabetes alters cardiac fibroblast activity in the myocardium in a 6-wk…

Current challenges in dedifferentiated fat cells research.

Dedifferentiated fat cells show great promises as a novel cell source for stem cell research. It has many advantages when used for cell-based therapeutics including abundance, pluripotency, and safety. However, there are many obstacles researchers…

Tags: *adult stem cells, *Biomedical Research, *Cell Dedifferentiation, *culture purity, *dedifferentiated fat cells, *dedifferentiation, *surface marker, 2016, Adipocytes/*cytology, Animals, Cells, Cultured, Evancho-Chapman M Michelle, George Richard L, Humans, Organogenesis, Phenotype, Shah Mickey, Zhang Ge

Histochemical analyses of tissue-engineered human menisci.

The field of tissue engineering remains one of the least explored areas of current meniscal research but holds great promise. In this investigation, meniscal fibrochondrocytes were isolated from fresh human meniscal tissue and seeded onto synthetic…

Bcl-2 positively regulates Sox9-dependent chondrocyte gene expression by suppressing the MEK-ERK1/2 signaling pathway.

Bcl-2 is an anti-apoptotic protein that has recently been shown to regulate other cellular functions. We previously reported that Bcl-2 regulates chondrocyte matrix gene expression, independent of its anti-apoptotic function. Here, we further…

Tags: *Gene Expression Regulation, 2005, Adenoviridae/genetics, Animals, Apoptosis, beta-Galactosidase/metabolism, Blotting, Butadienes/pharmacology, Caspase Inhibitors, Cell Differentiation, Cell Line, Chondrocytes/*metabolism, Collagen Type II/metabolism, Department of Anatomy & Neurobiology, Down-Regulation, Enzyme Inhibitors/pharmacology, Fibroblasts/metabolism, Fluorescence, Genetic, High Mobility Group Proteins/*metabolism, Horton Walter E Jr, Lac Operon, Luciferases/metabolism, MAP Kinase Kinase Kinases/*metabolism, McBurney Denise, Messenger/metabolism, Microscopy, Mitogen-Activated Protein Kinase 1/*metabolism, Mitogen-Activated Protein Kinase 3/*metabolism, NEOMED College of Medicine, NF-kappa B/metabolism, Nitriles/pharmacology, Phenotype, Phosphorylation, Promoter Regions, Protein Kinase C-alpha, Protein Kinase C/antagonists & inhibitors, Proteoglycans/metabolism, Proto-Oncogene Proteins c-bcl-2/*metabolism, Rats, Reverse Transcriptase Polymerase Chain Reaction, RNA, Signal Transduction, Small Interfering/metabolism, SOX9 Transcription Factor, Sprague-Dawley, The Journal of biological chemistry, Time Factors, Transcription, Transcription Factors/*metabolism, Transfection, Western, Yagi Rieko

Intrajoint comparisons of gene expression patterns in human osteoarthritis suggest a change in chondrocyte phenotype.

Osteoarthritis (OA) is a degenerative cartilage disease with varying degrees of severity within a given joint. The purpose of this study was to define a sampling procedure for comparing human minimal and advanced OA cartilage in the same patient and…

Lipopolysaccharide supports maintaining the stemness of CD133(+) hepatoma cells through activation of the NF-kappaB/HIF-1alpha pathway.

Due to the existence of cancer stem cells (CSCs), persistence and relapse of human hepatocellular carcinoma (HCC) are common after treatment with existing anti-cancer therapies. Emerging evidence indicates that lipopolysaccharide (LPS) plays a…

Tags: *Cancer stem cells, *Lipopolysaccharide, *Plasticity, *Stemness maintenance, *Tumor microenvironment, 2016, AC133 Antigen/genetics/*metabolism, alpha Subunit/genetics/*metabolism, Animals, Antineoplastic Agents – Pharmacodynamics, Antineoplastic Agents/pharmacology, Body Weights and Measures, Cancer letters, Carcinoma, Cell Line, Cell Movement – Drug Effects, Cell Movement/drug effects, Cell Physiology, Cell Physiology – Drug Effects, Cell Proliferation/drug effects, Department of Integrative Medical Sciences, Drug Resistance, Gene Expression Regulation, Genes, Genetic Techniques, Han Zhi-Peng, Hepatocellular, Hepatocellular – Drug Therapy, Hepatocellular – Metabolism, Hepatocellular – Pathology, Hepatocellular/drug therapy/genetics/*metabolism/pathology, Humans, Hypoxia-Inducible Factor 1, Inbred BALB C, Jing Ying-Ying, Lai Fo-Bao, Li Rong, Li Xiao-Yong, Lipopolysaccharides – Pharmacodynamics, Lipopolysaccharides/*pharmacology, Liu Jing-Feng, Liu Wen-Ting, Liver Neoplasms, Liver Neoplasms – Drug Therapy, Liver Neoplasms – Metabolism, Liver Neoplasms – Pathology, Liver Neoplasms/drug therapy/genetics/*metabolism/pathology, Male, Mice, NEOMED College of Medicine, Neoplasm, Neoplasm Invasiveness, Neoplastic, Neoplastic Stem Cells/*drug effects/metabolism/pathology, NF-kappa B – Metabolism, NF-kappa B/*metabolism, Nude, Pan Xiao-Rong, Phenotype, Proteins, Proteins – Metabolism, RNA Interference, Shi Rong-Yu, Signal Transduction – Drug Effects, Signal Transduction/drug effects, Stem Cells – Drug Effects, Stem Cells – Metabolism, Stem Cells – Pathology, Time Factors, Transfection, Tumor Burden, Tumor Microenvironment, Wei Li-Xin, Wu Meng-Chao, Yang Xue, Yu Guo-Feng, Zeng Jian-Xing, Zhang Hang-Jie, Zhang Ping, Zhao Qiu-Dong

Exacerbation of sensorimotor dysfunction in mice deficient in Atp13a2 and overexpressing human wildtype alpha-synuclein.

Loss of function mutations in the gene ATP13A2 are associated with Kufor-Rakeb Syndrome and Neuronal Ceroid Lipofuscinosis, the former designated as an inherited form of Parkinson's disease (PD). The function of ATP13A2 is unclear but in vitro…

Mutation in osteoactivin decreases bone formation in vivo and osteoblast differentiation in vitro.

We have previously identified osteoactivin (OA), encoded by Gpnmb, as an osteogenic factor that stimulates osteoblast differentiation in vitro. To elucidate the importance of OA in osteogenesis, we characterized the skeletal phenotype of a mouse…

Alignment of inducible vascular progenitor cells on a micro-bundle scaffold improves cardiac repair following myocardial infarction.

Ischemic heart disease is still the leading cause of death even with the advancement of pharmaceutical therapies and surgical procedures. Early vascularization in the ischemic heart is critical for a better outcome. Although stem cell therapy has…

Tags: *Cardiovascular regeneration, *Ischemic heart diseases, *Micro-bundle scaffold, *Myocardial infarction, *Neovascularization, *Stem cells, *Tissue Scaffolds, *Vascular progenitor cells, 2017, Animal, Animals, Basic research in cardiology, Cell Differentiation, Cell Proliferation, Cell Survival, Cells, Chilian William M, Coculture Techniques, Cultured, Cumpston Devan, Department of Integrative Medical Sciences, Disease Models, Dong Feng, Endothelial Progenitor Cells/metabolism/*transplantation, Enrick Molly, Fibroblast Growth Factor 2/metabolism, Hakobyan Tatev, Jamaiyar Anurag, Janota Danielle, Kolz Christopher L, Lactic Acid/*chemistry, Muscle, Myocardial Infarction/metabolism/pathology/physiopathology/*surgery, Myocardium/metabolism/*pathology, Myocytes, NEOMED College of Medicine, Newby Bi-Min Zhang, Ohanyan Vahagn, Paracrine Communication, Phenotype, Physiologic, Polyglycolic Acid/*chemistry, Polylactic Acid-Polyglycolic Acid Copolymer, Rats, Signal Transduction, Smooth, Smooth Muscle/metabolism/*transplantation, Song Hokyung, Sprague-Dawley, Stevanov Kelly, Time Factors, Tissue Engineering/*methods, Vascular Endothelial Growth Factor A/metabolism, Vascular/metabolism/*transplantation, Ventricular Remodeling, Wan Weiguo, Yin Liya

A novel role for Bcl-2 associated-athanogene-1 (Bag-1) in regulation of the endoplasmic reticulum stress response in mammalian chondrocytes.

BAG-1 (Bcl-2 associated athanogene-1) is a multifunctional protein, linking cell proliferation, cell death, protein folding, and cell stress. In vivo, BAG-1 is expressed in growth plate and articular cartilage, and the expression of BAG-1 is…

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