Hyperinsulinism in Infancy: From Basic Science to Clinical Disease

2004 ◽  
Vol 84 (1) ◽  
pp. 239-275 ◽  
Author(s):  
MARK J. DUNNE ◽  
KAREN E. COSGROVE ◽  
RUTH M. SHEPHERD ◽  
ALBERT AYNSLEY-GREEN ◽  
KEITH J. LINDLEY
Keyword(s):  
Ion Channel ◽  
Basic Science ◽  
Cell Biology ◽  
Clinical Medicine ◽  
Cell Metabolism ◽  
Cell Types ◽  
Clinical Disease ◽  
Molecular Physiology ◽  
Almost All ◽  
The Relationship

Dunne, Mark J., Karen E. Cosgrove, Ruth M. Shepherd, Albert Aynsley-Green, and Keith J. Lindley. Hyperinsulinism in Infancy: From Basic Science to Clinical Disease. Physiol Rev 84: 239–275, 2004; 10.1152/physrev.00022.2003.—Ion channelopathies have now been described in many well-characterized cell types including neurons, myocytes, epithelial cells, and endocrine cells. However, in only a few cases has the relationship between altered ion channel function, cell biology, and clinical disease been defined. Hyperinsulinism in infancy (HI) is a rare, potentially lethal condition of the newborn and early childhood. The causes of HI are varied and numerous, but in almost all cases they share a common target protein, the ATP-sensitive K+ channel. From gene defects in ion channel subunits to defects in β-cell metabolism and anaplerosis, this review describes the relationship between pathogenesis and clinical medicine. Until recently, HI was generally considered an orphan disease, but as parallel defects in ion channels, enzymes, and metabolic pathways also give rise to diabetes and impaired insulin release, the HI paradigm has wider implications for more common disorders of the endocrine pancreas and the molecular physiology of ion transport.

Silver Nanomaterials in Contemporary Molecular Physiology Research

2020 ◽  
Vol 27 (3) ◽  
pp. 411-422 ◽  
Author(s):  
Igor Pantic ◽  
David Sarenac ◽  
Mila Cetkovic ◽  
Milan Milisavljevic ◽  
Rastko Rakocevic ◽  
...  
Keyword(s):  
Silver Nanoparticles ◽  
Clinical Medicine ◽  
Chemical Properties ◽  
Short Review ◽  
Engineering Industry ◽  
Molecular Physiology ◽  
Potential Applications ◽  
Metallic Nanomaterials ◽  
Living Organisms ◽  
Almost All

Silver nanoparticles have numerous potential applications in engineering, industry, biology and medicine. Because of their unique chemical properties, they have become the focus of many research teams all over the world. Silver nanoparticles may exhibit significant antimicrobial and anticancer effects, and they may be a valuable part of various bioassays and biosensors. However, the research on biological and medical uses of AgNPs is related with numerous potential problems and challenges that need to be overcome in the years ahead. Possible toxic effects of silver nanoparticles on living organisms represent a great concern, both in clinical medicine and public health. Nevertheless, in the future, it may be expected that all metallic nanomaterials, including the ones made from silver will greatly benefit almost all natural scientific fields. In this short review, we focus on the recent research on silver nanoparticles in experimental physiology, as well as other areas of fundamental and clinical medicine.

scholarly journals The ACID PHOSPHATASE 1 regulates Pi stress adaptation by maintaining intracellular Pi homeostasis

2021 ◽  
Author(s):  
Su Deng ◽  
Jingyi Li ◽  
Zezhen Du ◽  
Zixuan Wu ◽  
Jian Yang ◽  
...  
Keyword(s):  
Acid Phosphatase ◽  
Cell Metabolism ◽  
Cell Types ◽  
Stress Conditions ◽  
Pi Homeostasis ◽  
Pi Starvation ◽  
Human Acid ◽  
Intracellular Phosphate ◽  
Almost All ◽  
Pi Stress

The concentration and homeostasis of intracellular phosphate (Pi) are crucial for sustaining cell metabolism and growth. During short-term Pi starvation, intracellular Pi is maintained relatively constant at the expense of vacuolar Pi. After the vacuolar stored Pi is exhausted, the plant cells induce the synthesis of intracellular acid phosphatase (APase) to recycle Pi from expendable organic phosphate (Po). In this study, the expression, enzymatic activity and subcellular localization of ACID PHOSPHATASE 1 (OsACP1) were determined. OsACP1 expression is specifically induced in almost all cell types of leaves and roots under Pi stress conditions. OsACP1 encodes an acid phosphatase with broad Po substrates and localizes in the endoplasmic reticulum (ER) and Golgi apparatus (GA). Phylogenic analysis demonstrates that OsACP1 has a similar structure with human acid phosphatase PHOSPHO1. Overexpression or mutation of OsACP1 affected Po degradation and utilization, which further influenced plant growth and productivity under both Pi-sufficient and Pi-deficient conditions. Moreover, overexpression of OsACP1 significantly affected intracellular Pi homeostasis and Pi starvation signalling. We concluded that OsACP1 is an active acid phosphatase that regulates rice growth under Pi stress conditions by recycling Pi from Po in the ER and GA.

scholarly journals Chemical and Genetic Engineering of Selective Ion Channel–Ligand Interactions

Science ◽  
2011 ◽  
Vol 333 (6047) ◽  
pp. 1292-1296 ◽  
Author(s):  
Christopher J. Magnus ◽  
Peter H. Lee ◽  
Deniz Atasoy ◽  
Helen H. Su ◽  
Loren L. Looger ◽  
...  
Keyword(s):  
Ion Channel ◽  
Cell Types ◽  
Neuron Activity ◽  
Ionic Flux ◽  
Ion Conductance ◽  
Ionic Conductances ◽  
Ligand Interactions ◽  
Cell Type Specific ◽  
The Relationship

Ionic flux mediates essential physiological and behavioral functions in defined cell populations. Cell type–specific activators of diverse ionic conductances are needed for probing these effects. We combined chemistry and protein engineering to enable the systematic creation of a toolbox of ligand-gated ion channels (LGICs) with orthogonal pharmacologic selectivity and divergent functional properties. The LGICs and their small-molecule effectors were able to activate a range of ionic conductances in genetically specified cell types. LGICs constructed for neuronal perturbation could be used to selectively manipulate neuron activity in mammalian brains in vivo. The diversity of ion channel tools accessible from this approach will be useful for examining the relationship between neuronal activity and animal behavior, as well as for cell biological and physiological applications requiring chemical control of ion conductance.

Contemporary Qur'anic Studies in Iran and its Relationship with Qur'anic Studies in the West

2012 ◽  
Vol 14 (1) ◽  
pp. 45-72
Author(s):  
Morteza Karimi-Nia
Keyword(s):  
Islamic Law ◽  
Human Sciences ◽  
The West ◽  
Communications Technologies ◽  
Religious Learning ◽  
The Status ◽  
Islamic Republic Of Iran ◽  
Almost All ◽  
The Impact ◽  
The Relationship

The status of tafsīr and Qur'anic studies in the Islamic Republic of Iran has changed significantly during recent decades. The essay provides an overview of the state of Qur'anic studies in Iran today, aiming to examine the extent of the impact of studies by Western scholars on Iranian academic circles during the last three decades and the relationship between them. As in most Islamic countries, the major bulk of academic activity in Iran in this field used to be undertaken by the traditional ʿulamāʾ; however, since the beginning of the twentieth century and the establishment of universities and other academic institutions in the Islamic world, there has been increasing diversity and development. After the Islamic Revolution, many gradual changes in the structure and approach of centres of religious learning and universities have occurred. Contemporary advancements in modern sciences and communications technologies have gradually brought the institutions engaged in the study of human sciences to confront the new context. As a result, the traditional Shīʿī centres of learning, which until 50 years ago devoted themselves exclusively to the study of Islamic law and jurisprudence, today pay attention to the teaching of foreign languages, Qur'anic sciences and exegesis, including Western studies about the Qur'an, to a certain extent, and recognise the importance of almost all of the human sciences of the West.

scholarly journals Mitochondrial Glucocorticoid Receptors and Their Actions

2021 ◽  
Vol 22 (11) ◽  
pp. 6054
Author(s):  
Ioanna Kokkinopoulou ◽  
Paraskevi Moutsatsou
Keyword(s):  
Gene Expression ◽  
Glucocorticoid Receptors ◽  
Mitochondrial Gene ◽  
Cell Types ◽  
Ros Generation ◽  
Mitochondrial Gene Expression ◽  
Mitochondrial Energy Metabolism ◽  
Bcl2 Family ◽  
Cellular Processes ◽  
Almost All

Mitochondria are membrane organelles present in almost all eukaryotic cells. In addition to their well-known role in energy production, mitochondria regulate central cellular processes, including calcium homeostasis, Reactive Oxygen Species (ROS) generation, cell death, thermogenesis, and biosynthesis of lipids, nucleic acids, and steroid hormones. Glucocorticoids (GCs) regulate the mitochondrially encoded oxidative phosphorylation gene expression and mitochondrial energy metabolism. The identification of Glucocorticoid Response Elements (GREs) in mitochondrial sequences and the detection of Glucocorticoid Receptor (GR) in mitochondria of different cell types gave support to hypothesis that mitochondrial GR directly regulates mitochondrial gene expression. Numerous studies have revealed changes in mitochondrial gene expression alongside with GR import/export in mitochondria, confirming the direct effects of GCs on mitochondrial genome. Further evidence has made clear that mitochondrial GR is involved in mitochondrial function and apoptosis-mediated processes, through interacting or altering the distribution of Bcl2 family members. Even though its exact translocation mechanisms remain unknown, data have shown that GR chaperones (Hsp70/90, Bag-1, FKBP51), the anti-apoptotic protein Bcl-2, the HDAC6- mediated deacetylation and the outer mitochondrial translocation complexes (Tom complexes) co-ordinate GR mitochondrial trafficking. A role of mitochondrial GR in stress and depression as well as in lung and hepatic inflammation has also been demonstrated.

scholarly journals Protein tyrosine phosphatase receptor type C (PTPRC or CD45)

2021 ◽  
pp. jclinpath-2020-206927
Author(s):  
Maryam Ahmed Al Barashdi ◽  
Ahlam Ali ◽  
Mary Frances McMullin ◽  
Ken Mills
Keyword(s):  
Protein Tyrosine Phosphatase ◽  
Protein Tyrosine Kinase ◽  
Biological Activities ◽  
Tyrosine Phosphatase ◽  
Cell Types ◽  
Receptor Type ◽  
Future Research ◽  
Protein Tyrosine ◽  
Type C ◽  
Almost All

The leucocyte common antigen, protein tyrosine phosphatase receptor type C (PTPRC), also known as CD45, is a transmembrane glycoprotein, expressed on almost all haematopoietic cells except for mature erythrocytes, and is an essential regulator of T and B cell antigen receptor-mediated activation. Disruption of the equilibrium between protein tyrosine kinase and phosphatase activity (from CD45 and others) can result in immunodeficiency, autoimmunity, or malignancy. CD45 is normally present on the cell surface, therefore it works upstream of a large signalling network which differs between cell types, and thus the effects of CD45 on these cells are also different. However, it is becoming clear that CD45 plays an essential role in the innate immune system and this is likely to be a key area for future research. In this review of PTPRC (CD45), its structure and biological activities as well as abnormal expression of CD45 in leukaemia and lymphoma will be discussed.

scholarly journals Double parton distributions in the pion from lattice QCD

2021 ◽  
Vol 2021 (2) ◽  
Author(s):  
Gunnar S. Bali ◽  
Luca Castagnini ◽  
Markus Diehl ◽  
Jonathan R. Gaunt ◽  
Benjamin Gläßle ◽  
...  
Keyword(s):  
Spatial Distribution ◽  
Lattice Qcd ◽  
Quark Mass ◽  
Matrix Elements ◽  
Parton Distributions ◽  
Almost All ◽  
The Relationship

Abstract We perform a lattice study of double parton distributions in the pion, using the relationship between their Mellin moments and pion matrix elements of two local currents. A good statistical signal is obtained for almost all relevant Wick contractions. We investigate correlations in the spatial distribution of two partons in the pion, as well as correlations involving the parton polarisation. The patterns we observe depend significantly on the quark mass. We investigate the assumption that double parton distributions approximately factorise into a convolution of single parton distributions.

scholarly journals Interactome Analysis of KIN (Kin17) Shows New Functions of This Protein

2021 ◽  
Vol 43 (2) ◽  
pp. 767-781
Author(s):  
Vanessa Pinatto Gaspar ◽  
Anelise Cardoso Ramos ◽  
Philippe Cloutier ◽  
José Renato Pattaro Junior ◽  
Francisco Ferreira Duarte Junior ◽  
...  
Keyword(s):  
Dna Replication ◽  
Protein Interactions ◽  
Ribosome Biogenesis ◽  
Cell Metabolism ◽  
Cell Types ◽  
Protein Protein Interactions ◽  
Cellular Mechanisms ◽  
Cancerous Cell ◽  
First Time

KIN (Kin17) protein is overexpressed in a number of cancerous cell lines, and is therefore considered a possible cancer biomarker. It is a well-conserved protein across eukaryotes and is ubiquitously expressed in all cell types studied, suggesting an important role in the maintenance of basic cellular function which is yet to be well determined. Early studies on KIN suggested that this nuclear protein plays a role in cellular mechanisms such as DNA replication and/or repair; however, its association with chromatin depends on its methylation state. In order to provide a better understanding of the cellular role of this protein, we investigated its interactome by proximity-dependent biotin identification coupled to mass spectrometry (BioID-MS), used for identification of protein–protein interactions. Our analyses detected interaction with a novel set of proteins and reinforced previous observations linking KIN to factors involved in RNA processing, notably pre-mRNA splicing and ribosome biogenesis. However, little evidence supports that this protein is directly coupled to DNA replication and/or repair processes, as previously suggested. Furthermore, a novel interaction was observed with PRMT7 (protein arginine methyltransferase 7) and we demonstrated that KIN is modified by this enzyme. This interactome analysis indicates that KIN is associated with several cell metabolism functions, and shows for the first time an association with ribosome biogenesis, suggesting that KIN is likely a moonlight protein.

scholarly journals Morphological and Ultrastructural Characterization of Hemocytes in an Insect Model, the Hematophagous Dipetalogaster maxima (Hemiptera: Reduviidae)

Insects ◽  
2021 ◽  
Vol 12 (7) ◽  
pp. 640
Author(s):  
Natalia R. Moyetta ◽  
Fabián O. Ramos ◽  
Jimena Leyria ◽  
Lilián E. Canavoso ◽  
Leonardo L. Fruttero
Keyword(s):  
Cell Biology ◽  
Cell Types ◽  
Transmission Electron ◽  
Ultrastructural Characterization ◽  
Current Classification ◽  
Contrast Microscopy ◽  
First Time ◽  
Controversial Aspect

Hemocytes, the cells present in the hemolymph of insects and other invertebrates, perform several physiological functions, including innate immunity. The current classification of hemocyte types is based mostly on morphological features; however, divergences have emerged among specialists in triatomines, the insect vectors of Chagas’ disease (Hemiptera: Reduviidae). Here, we have combined technical approaches in order to characterize the hemocytes from fifth instar nymphs of the triatomine Dipetalogaster maxima. Moreover, in this work we describe, for the first time, the ultrastructural features of D. maxima hemocytes. Using phase contrast microscopy of fresh preparations, five hemocyte populations were identified and further characterized by immunofluorescence, flow cytometry and transmission electron microscopy. The plasmatocytes and the granulocytes were the most abundant cell types, although prohemocytes, adipohemocytes and oenocytes were also found. This work sheds light on a controversial aspect of triatomine cell biology and physiology setting the basis for future in-depth studies directed to address hemocyte classification using non-microscopy-based markers.

scholarly journals Bayonet-shaped language development in autism with regression: a retrospective study

2021 ◽  
Vol 12 (1) ◽  
Author(s):  
David Gagnon ◽  
Abderrahim Zeribi ◽  
Élise Douard ◽  
Valérie Courchesne ◽  
Borja Rodríguez-Herreros ◽  
...  
Keyword(s):  
Language Development ◽  
Linear Models ◽  
Autism Spectrum ◽  
Early Language ◽  
Verbal Iq ◽  
Logistic Regression Models ◽  
Language Level ◽  
Language Outcome ◽  
Almost All ◽  
The Relationship

Abstract Background Language delay is one of the major referral criteria for an autism evaluation. Once an autism spectrum diagnosis is established, the language prognosis is among the main parental concerns. Early language regression (ELR) is observed by 10–50% of parents but its relevance to late language level and socio-communicative ability is uncertain. This study aimed to establish the predictive value of ELR on the progression of language development and socio-communicative outcomes to guide clinicians in addressing parents’ concerns at the time of diagnosis. Methods We used socio-communicative, language, and cognitive data of 2,047 autism spectrum participants from the Simons Simplex Collection, aged 4–18 years (mean = 9 years; SD = 3.6). Cox proportional hazard and logistic regression models were used to evaluate the effect of ELR on language milestones and the probability of using complex and flexible language, as defined by the choice of ADOS module at enrollment. Linear models were then used to evaluate the relationship of ELR and non-verbal IQ with socio-communicative and language levels. Results ELR is associated with earlier language milestones but delayed attainment of fluent, complex, and flexible language. However, this language outcome can be expected for almost all autistic children without intellectual disability at 18 years of age. It is mostly influenced by non-verbal IQ, not ELR. The language and socio-communicative level of participants with flexible language, as measured by the Vineland and ADOS socio-communicative subscales, was not affected by ELR. Limitations This study is based on a relatively coarse measure of ultimate language level and relies on retrospective reporting of early language milestones and ELR. It does not prospectively document the age at which language catches up, the relationship between ELR and other behavioral areas of regression, nor the effects of intervention. Conclusions For autistic individuals with ELR and a normal level of non-verbal intelligence, language development follows a “bayonet shape” trajectory: early first words followed by regression, a plateau with limited progress, and then language catch up.

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