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Ahead Of Their Time: The Story Of Alice Berry Graham And Katharine Berry Richardson, The Founders Of Children's Mercy Hospital In Kansas City, Jane F. Knapp Md, Robert Schremmer

Posters

Describes the founding of Children's Mercy Hospital through the story of its founders, Katharine Berry Richardson and Alice Berry Graham.

Development And Evolution Of Risk Assessment For Food Allergens, Rene W. R. Crevel, Joseph Baumert, Athanasia Baka, Geert Houben, André C. Knulst, Astrid Kruizinga, Stefano Luccioli, Stephen L. Taylor, Charlotte B. Madsen

Department of Food Science and Technology: Faculty Publications

The need to assess the risk from food allergens derives directly from the need to manage effectively this food safety hazard. Work spanning the last two decades dispelled the initial thinking that food allergens were so unique that the risk they posed was not amenable to established risk assessment approaches and methodologies. Food allergens possess some unique characteristics, which make a simple safety assessment approach based on the establishment of absolute population thresholds inadequate. Dose distribution modelling of MEDs permitted the quantification of the risk of reaction at the population level and has been readily integrated with consumption and contamination …

Cell- And Virus-Mediated Regulation Of The Barrier-To-Autointegration Factor’S Phosphorylation State Controls Its Dna Binding, Dimerization, Subcellular Localization, And Antipoxviral Activity, Augusta Jamin, April Wicklund, Matthew S. Wiebe

Nebraska Center for Virology: Faculty Publications

Barrier-to-autointegration factor (BAF) is a DNA binding protein with multiple cellular functions, including the ability to act as a potent defense against vaccinia virus infection. This antiviral function involves BAF’s ability to condense double-stranded DNA and subsequently prevent viral DNA replication. In recent years, it has become increasingly evident that dynamic phosphorylation involving the vaccinia virus B1 kinase and cellular enzymes is likely a key regulator of multiple BAF functions; however, the precise mechanisms are poorly understood. Here we analyzed how phosphorylation impacts BAF’s DNA binding, subcellular localization, dimerization, and antipoxviral activity through the characterization of BAF phosphomimetic and unphosphorylatable …

Live Siv Vaccine Correlate Of Protection: Immune Complex-Inhibitory Fc Receptor Interactions That Reduce Target Cell Availability, Anthony J. Smith, Stephen W. Wietgrefe, Liang Shang, Cavan S. Reilly, Peter J. Southern, Katherine E. Perkey, Lijie Duan, Heinz Kohler, Sybille Muller, James Robinson, John V. Carlis, Qingsheng Li, R. Paul Johnson, Ashley T. Haase

Nebraska Center for Virology: Faculty Publications

Principles to guide design of an effective vaccine against HIV are greatly needed, particularly to

protect women in the pandemic’s epicentre in Africa. We have been seeking these principles by

identifying correlates of the robust protection associated with SIV_mac239Δnef vaccination in the

SIV-rhesus macaque animal model of HIV-1 transmission to women. We have identified one

correlate of SIV_mac239Δnef protection against vaginal challenge as a resident mucosal system for

SIV-gp41 trimer antibody production and neonatal Fc receptor (FcRn)-mediated concentration of

these antibodies on the path of virus entry to inhibit establishment of infected founder populations

at the …

Review Of Epidemiology And Transmission Of Kaposi’S Sarcoma-Associated Herpesvirus, Veenu Minhas, Charles Wood

Nebraska Center for Virology: Faculty Publications

This review summarizes the current knowledge pertaining to Kaposi sarcoma-associated herpesvirus (KSHV) epidemiology and transmission. Since the identification of KSHV twenty years ago, it is now known to be associated with Kaposi’s sarcoma (KS), primary effusion lymphoma, and multicentric Castleman’s disease. Many studies have been conducted to understand its epidemiology and pathogenesis and their results clearly show that the worldwide distribution of KSHV is uneven. Some geographical areas, such as sub-Saharan Africa, the Mediterranean region and the Xinjiang region of China, are endemic areas, but Western Europe and United States have a low prevalence in the general population. This makes …

Vaccines Within Vaccines: The Use Of Adenovirus Types 4 And 7 As Influenza Vaccine Vectors, Eric A. Weaver

Nebraska Center for Virology: Faculty Publications

adenovirus Types 4 and 7 (ad4 and ad7) are associated with acute respiratory distress (aRD). In order to prevent wide- spread ad-associated aRD (ad-aRD) the United states military immunizes new recruits using a safe and effective lyophi- lized wildtype ad4 and ad7 delivered orally in an enteric-coated capsule. We cloned ad4 and ad7 and modified them to express either a GFP-Luciferase (GFPLuc) fusion gene or a centralized influenza H1 hemagglutinin (Ha1-con). BaLB/c mice were injected with GFPLuc expressing viruses intramuscularly (i.m.) and intranasally (i.n.). ad4 induced significantly higher luciferase expression levels as compared with ad7 by both routes. ad7 transduction …

Sulfites, Steve L. Taylor, Robert K. Bush, Julie A. Nordlee

Department of Food Science and Technology: Faculty Publications

Key Concepts

Sulfites are frequently used food and drug additives.

Ingestion of sulfite residues has been documented to trigger asthmatic reactions in sensitive individuals.

Sulfite-induced asthma occurs in less than 5% of asthmatic individuals, and those with severe, persistent asthma are at greatest risk.

The diagnosis of sulfite-induced asthma is best made by blinded oral challenge with assessment of lung function.

Labeling regulations in the United States alert sulfite-sensitive individuals to the presence of sulfites in foods, which must then be avoided.

Environmental And Occupational Factors Associated With Chronic Myeloid Leukemia: A Case-Control Study, R. Kasmani, N.A. Othieno-Abinya, M.T. Singh Riyat, G.W. Kiarie, Peter Wanzala

Pathology, East Africa

Background: The relationship between chronic myeloid leukemia (CML) and a broad range of exposures to occupational and environmental factors known to cause leukemia in general is limited. CML is by and large incurable and treatment is just palliative and life prolonging, with high case fatality rate, even in the best centers. Furthermore treatment is very expensive. Identification of leukemogenic factors is therefore important if this can lead to simple public health interventions.

Objective: The objective was to determine key environmental and occupational exposure factors that may be associated with CML.

Methods: This was a case-control study involving CML …

Fluid Flow Shear Stress Over Podocytes Is Increased In The Solitary Kidney., Tarak Srivastava, Gianni E. Celsi, Mukut Sharma, Hongying Dai, Ellen T. Mccarthy, Melanie Ruiz, Patricia A. Cudmore, Uri S. Alon, Ram Sharma, Virginia A. Savin

Manuscripts, Articles, Book Chapters and Other Papers

BACKGROUND: Glomerular hyperfiltration is emerging as the key risk factor for progression of chronic kidney disease (CKD). Podocytes are exposed to fluid flow shear stress (FFSS) caused by the flow of ultrafiltrate within Bowman's space. The mechanism of hyperfiltration-induced podocyte injury is not clear. We postulated that glomerular hyperfiltration in solitary kidney increases FFSS over podocytes.

METHODS: Infant Sprague-Dawley rats at 5 days of age and C57BL/6J 14-week-old adult mice underwent unilateral nephrectomy. Micropuncture and morphological studies were then performed on 20- and 60-day-old rats. FFSS over podocytes in uninephrectomized rats and mice was calculated using the recently published equation …

Analysis Of The Role Of Astrocyte Elevated Gene-1 In Normal Liver Physiology And In The Onset And Progression Of Hepatocellular Carcinoma, Chadia L. Robertson

Theses and Dissertations

First identified over a decade ago, Astrocyte Elevated Gene-1 (AEG-1) has been studied extensively due to early reports of its overexpression in various cancer cell lines. Research groups all over the globe including our own have since identified AEG-1 overexpression in cancers of diverse lineages including cancers of the liver, colon, skin, prostate, breast, lung, esophagus, neurons and neuronal glia as compared to matched normal tissue. A comprehensive and convincing body of data currently points to AEG-1 as an essential component, critical to the progression and perhaps onset of cancer. AEG-1 is a potent activator of multiple pro-tumorigenic signal transduction …

Perceptions Of Acupuncture And Acupressure By Anesthesia Providers, Amanda Faircloth

Theses and Dissertations

BACKGROUND: Randomized controlled trials show acupuncture and acupressure support anesthesia management by decreasing anxiety, opioid requirements and treating post-operative nausea and vomiting. Acupuncture and acupressure have demonstrated clinical usefulness and received governmental support (NIH, PPACA, WHO, U.S. Military), but have not yet diffused into mainstream anesthesia practice. This study examined US anesthesia providers' perceptions of acupuncture and acupressure.

METHODS: Ninety-six anesthesiology departments stratified by geographic region (Northeast, South, West, and Midwest) and institution type (university medical centers, community hospitals, children’s hospitals, and VA hospitals) were selected for participation in an anonymous, online survey. The target sample was 1,728 providers …

International Standards For Fetal Growth Based On Serial Ultrasound Measurements: The Fetal Growth Longitudinal Study Of The Intergrowth-21st Project, Aris T. Papageorghiou, Eric O. Ohuma, Douglas G. Altman, Tullia Todros, Leila Cheikh Ismail, Ann Lambert, Yasmin A. Jaffer, Enrico Bertino, Michael G. Gravett, J Alison Noble, Ruyan Pang, Cesar G. Victora, Fernando C. Barros, Maria Carvalho, Laurent J. Salomon, Zulfiqar A. Bhutta, Stephen H. Kennedy, José Villar

Obstetrics and Gynaecology, East Africa

Background: In 2006, WHO produced international growth standards for infants and children up to age 5 years on the basis of recommendations from a WHO expert committee. Using the same methods and conceptual approach, the Fetal Growth Longitudinal Study (FGLS), part of the INTERGROWTH-21st Project, aimed to develop international growth and size standards for fetuses.

Methods: The multicentre, population-based FGLS assessed fetal growth in geographically defined urban populations in eight countries, in which most of the health and nutritional needs of mothers were met and adequate antenatal care was provided. We used ultrasound to take fetal anthropometric measurements prospectively from …

Pregnancy Outcomes In Benghazi, Libya, Before And During The Armed Conflict In 2011, Z. Bodalal, K. Agnaeber, N. Nagelkerke, B. Stirling, Marleen Temmerman, O. Degomme

Obstetrics and Gynaecology, East Africa

Stressful life events experienced by pregnant women may lead to adverse obstetric outcomes. This study in Benghazi compared the rates of preterm, low-birth-weight and caesarean-section births at Al-Jamhouria hospital in the months before and during the armed conflict in Libya in 2011. Data were collected on all women admitted to the delivery ward during February to May 2011 (the months of the most active fighting in the city) (n = 7096), and October to December 2010 (the months immediately before the war) (n = 5935). Compared with the preceding months there was a significant rise during the conflict in the …

The Symptom Experience Of Patients Receiving Epidermal Growth Factor Receptor Inhibitors, Josephine Ann Howard-Ruben

Dissertations

This study explored the symptom experience of patients receiving epidermal growth factor receptor inhibitors (EGFRIs) for breast, colon, head and neck, and colon cancer. EGFRIs are targeted therapies used at various points along the treatment continuum for these solid tumors, and may be first, second or third-line agents which can be used as single agents or in combination with other therapies. The most common side effect of these agents include dermatologic effects, such as rashes, hair, and nail changes, but they can also contribute to other side effects such as fatigue, anxiety and diarrhea. Most previous work has addressed the …

Using Verbal Autopsy To Measure Causes Of Death: The Comparative Performance Of Existing Methods, C.J.L. Mahende, R. Lozano, A.D. Flaxman, P. Serina, D. Phillips, A. Stewart, S.L. James, A. Vahdatpour, C. Atkinson, M.K. Freeman, S.L. Ohno, R. Black, S.M. Ali, A.H. Baqui, L. Dandona, E. Dantzer, G.L. Darmstadt, V. Das, U. Dhingra, A. Dutta, W. Fawzi, S. Gomez, B. Hernandez, R. Joshi, H.D. Kalter, A. Kumar, V. Kumar, M. Lucero, S. Mehta, Zul Premji, D. Ramirez-Villalobos, H. Remolador, I. Riley, M. Romero, M. Said, D. Sanvictores, S. Sazawal, V. Tallo, A. D. Lopez

Pathology, East Africa

Background: Monitoring progress with disease and injury reduction in many populations will require widespread use of verbal autopsy (VA). Multiple methods have been developed for assigning cause of death from a VA but their application is restricted by uncertainty about their reliability.

Methods: We investigated the validity of five automated VA methods for assigning cause of death: InterVA-4, Random Forest (RF), Simplified Symptom Pattern (SSP), Tariff method (Tariff), and King-Lu (KL), in addition to physician review of VA forms (PCVA), based on 12,535 cases from diverse populations for which the true cause of death had been reliably established. For adults, …

Harnessing Microbiome And Probiotic Research In Sub-Saharan Africa: Recommendations From An African Workshop, Gregor Reid, Nicholas Nduti, Wilbert Sybesma, Remco Kort, Tobias R. Kollmann, Rodney Adam, Hamadi Boga, Eric M. Brown, Alexandra Einerhand, Hani El-Nezami, Gregory B. Gloor, Irene I. Kavere, Johanna Lindahl, Amee Manges, Wondu Mamo, Rocio Martin, Amy Mcmillan, Jael Obiero, Pamela A. Ochieng’, Arnold Onyango, Stephen Rulisa, Eeva Salminen, Seppo Salminen, Antony Sije, Jonathan R. Swann, William Van Treuren, Daniel Waweru, Steve J. Kemp

Pathology, East Africa

To augment capacity-building for microbiome and probiotic research in Africa, a workshop was held in Nairobi, Kenya, at which researchers discussed human, animal, insect, and agricultural microbiome and probiotics/prebiotics topics. Five recommendations were made to promote future basic and translational research that benefits Africans.

Novel Therapeutic Strategies For Pancreatic Cancer, Bridget A. Quinn

Theses and Dissertations

Pancreatic cancer is a devastating disease that leaves patients with a very poor prognosis and few therapeutic options. Many of the treatment options available are the same that have been used for almost 2 decades. There is a dire need for both novel treatments for this disease as well as novel strategies of treatment. This body of work will introduce and provide evidence in support of a novel combination therapy for pancreatic cancer treatment, a novel strategy of modifying currently used chemotherapeutics for pancreatic cancer therapy, and a novel transgenic preclinical mouse model of pancreatic cancer. Sabutoclax, an antagonist of …

Culturable Aerobic And Facultative Anaerobic Intestinal Bacterial Flora Of Black Cobra (Naja Naja Karachiensis) In Southern Pakistan, Junaid Iqbal, Mehwish Sagheer, Nazneen Tabassum, Ruqaiyyah Siddiqui, Naveed Ahmed Khan

Department of Biological & Biomedical Sciences

Using morphological analysis and biochemical testing, here for the first time, we determined the culturable gut bacterial flora (aerobes and facultative anaerobes) in the venomous Black Cobra (Naja naja karachiensis) from South Asia. The findings revealed that these snakes inhabit potentially pathogenic bacteria including Serratia marcescens, Pseudomonas aeruginosa, Shewanella putrefaciens, Aeromonas hydrophila, Salmonella sp., Moraxella sp., Bacillus sp., Ochrobactrum anthropi, and Providencia rettgeri. These findings are of concern, as injury from snake bite can result in wound infections and tissue necrosis leading to sepsis/necrotizing fasciitis and/or expose consumers of snake meat/medicine in the community to infections.

Effect Of L-Type Calcium Channel Blocker (Amlodipine) On Myocardial Iron Deposition In Patients With Thalassaemia With Moderate-To-Severe Myocardial Iron Deposition: Protocol For A Randomised, Controlled Trial, Amarah Shakoor, Maaman Zahoor, Alina Sadaf, Najveen Alvi, Zehra Fadoo, Arjumand Rizvi, Farheen Quadri, Fateh Ali Tipoo, Mohammad Khurshid, Zaffar Sajjad, Steven Colan, Babar S Hasan

Department of Paediatrics and Child Health

Introduction: Sideroblastic cardiomyopathy secondary to repeated blood transfusions is a feared complication in thalassaemia. Control of myocardial iron is thus becoming the cornerstone of thalassaemia management. Recent evidence suggests a role for L-type Ca²⁺ channels in mediating iron uptake by the heart. Blocking the cellular iron uptake through these channels may add to the benefit of therapy to standard chelation in reducing myocardial iron. We aim to determine the efficacy of amlodipine (a calcium channel blocker) as an adjunct to standard aggressive chelation in retarding myocardial iron deposition in thalassaemics with or without cardiomyopathy.
Outcomes: The primary outcome …

Reaching Perinatal Women Online: The Healthy You, Healthy Baby Website And App, Lydia Hearn, Margaret Miller, Leanne Lester

Research outputs 2014 to 2021

Overwhelming evidence reveals the close link between unwarranted weight gain among childbearing women and childhood adiposity. Yet current barriers limit the capacity of perinatal health care providers (PHCPs) to offer healthy lifestyle counselling. In response, today's Internet savvy women are turning to online resources to access health information, with the potential of revolutionising health services by enabling PHCPs to guide women to appropriate online resources. This paper presents the findings of a project designed to develop an online resource to promote healthy lifestyles during the perinatal period. The methodology involved focus groups and interviews with perinatal women and PHCPs to …

Network Analysis Of Circular Permutations In Multidomain Proteins Reveals Functional Linkages For Uncharacterized Proteins., Donald Adjeroh, Yue Jiang, Bing-Hua Jiang, Jie Lin

Department of Pathology, Anatomy, and Cell Biology Faculty Papers

Various studies have implicated different multidomain proteins in cancer. However, there has been little or no detailed study on the role of circular multidomain proteins in the general problem of cancer or on specific cancer types. This work represents an initial attempt at investigating the potential for predicting linkages between known cancer-associated proteins with uncharacterized or hypothetical multidomain proteins, based primarily on circular permutation (CP) relationships. First, we propose an efficient algorithm for rapid identification of both exact and approximate CPs in multidomain proteins. Using the circular relations identified, we construct networks between multidomain proteins, based on which we perform …

Obesity Reduces Left Ventricular Strains, Torsion, And Synchrony In Mouse Models: A Cine Displacement Encoding With Stimulated Echoes (Dense) Cardiovascular Magnetic Resonance Study, Sage P. Kramer, David K. Powell, Christopher M. Haggerty, Cassi M. Binkley, Andrea C. Mattingly, Lisa A. Cassis, Frederick H. Epstein, Brandon K. Fornwalt

Pediatrics Faculty Publications

BACKGROUND: Obesity affects a third of adults in the US and results in an increased risk of cardiovascular mortality. While the mechanisms underlying this increased risk are not well understood, animal models of obesity have shown direct effects on the heart such as steatosis and fibrosis, which may affect cardiac function. However, the effect of obesity on cardiac function in animal models is not well-defined. We hypothesized that diet-induced obesity in mice reduces strain, torsion, and synchrony in the left ventricle (LV).

METHODS: Ten 12-week-old C57BL/6 J mice were randomized to a high-fat or low-fat diet. After 5 months on …

Sphingosine-1-Phosphate-Mediated Mobilization Of Hematopoietic Stem/Progenitor Cells During Intravascular Hemolysis Requires Attenuation Of Sdf-1-Cxcr4 Retention Signaling In Bone Marrow, Kasia Mierzejewska, Yuri M. Klyachkin, Janina Ratajczak, Ahmed Abdel-Latif, Magda Kucia, Mariusz Z. Ratajczak

Saha Cardiovascular Research Center Faculty Publications

Sphingosine-1-phosphate (S1P) is a crucial chemotactic factor in peripheral blood (PB) involved in the mobilization process and egress of hematopoietic stem/progenitor cells (HSPCs) from bone marrow (BM). Since S1P is present at high levels in erythrocytes, one might assume that, by increasing the plasma S1P level, the hemolysis of red blood cells would induce mobilization of HSPCs. To test this assumption, we induced hemolysis in mice by employing phenylhydrazine (PHZ). We observed that doubling the S1P level in PB from damaged erythrocytes induced only a marginally increased level of mobilization. However, if mice were exposed to PHZ together with the …

Inflammatory Cytokine Gene Expression In Mesenteric Adipose Tissue During Acute Experimental Colitis, William Conan Mustain, Marlene E. Starr, Joseph Daniel Valentino, Donald A. Cohen, Daiki Okamura, Chi Wang, B. Mark Evers, Hiroshi Saito

Markey Cancer Center Faculty Publications

BACKGROUND: Production of inflammatory cytokines by mesenteric adipose tissue (MAT) has been implicated in the pathogenesis of inflammatory bowel disease (IBD). Animal models of colitis have demonstrated inflammatory changes within MAT, but it is unclear if these changes occur in isolation or as part of a systemic adipose tissue response. It is also unknown what cell types are responsible for cytokine production within MAT. The present study was designed to determine whether cytokine production by MAT during experimental colitis is depot-specific, and also to identify the source of cytokine production within MAT.

METHODS: Experimental colitis was induced in 6-month-old C57BL/6 …

Using Expression Profiling To Understand The Effects Of Chronic Cadmium Exposure On Mcf-7 Breast Cancer Cells, Zelmina Lubovac-Pilav, Daniel M. Borras, Esmeralda Ponce, Maggie Louie

Collected Faculty Scholarship

Cadmium is a metalloestrogen known to activate the estrogen receptor and promote breast cancer cell growth. Previous studies have implicated cadmium in the development of more malignant tumors; however the molecular mechanisms behind this cadmium-induced malignancy remain elusive. Using clonal cell lines derived from exposing breast cancer cells to cadmium for over 6 months (MCF-7-Cd4, -Cd6, -Cd7, -Cd8 and -Cd12), this study aims to identify gene expression signatures associated with chronic cadmium exposure. Our results demonstrate that prolonged cadmium exposure does not merely result in the deregulation of genes but actually leads to a distinctive expression profile. The genes deregulated …

Surgery For Post-Vitrectomy Cataract, Diana V. Do, Stephen Gichuhi, Satyanarayana S. Vedula, Barbara S. Hawkins

Journal Articles: Ophthalmology

BACKGROUND: Cataract formation or acceleration can occur after intraocular surgery, especially following vitrectomy, a surgical technique for removing the vitreous which is used in the treatment of disorders that affect the posterior segment of the eye. The underlying problem that led to vitrectomy may limit the benefit from cataract surgery.

OBJECTIVES: The objective of this review was to evaluate the effectiveness and safety of surgery for post-vitrectomy cataract with respect to visual acuity, quality of life, and other outcomes.

SEARCH METHODS: We searched CENTRAL (which contains the Cochrane Eyes and Vision Group Trials Register) (The Cochrane Library 2013, Issue 4), …

Adiponectin Inhibits Oxidative/Nitrative Stress During Myocardial Ischemia And Reperfusion Via Pka Signaling., Yanqing Zhang, Xiao-Liang Wang, Jianli Zhao, Ya-Jing Wang, Wayne Bond Lau, Yue-Xing Yuan, Er-He Gao, Walter J. Koch, Xin-Liang Ma

Department of Emergency Medicine Faculty Papers

The cardioprotective effects of adiponectin (APN) against myocardial ischemia/reperfusion (MI/R) injury are well known. However, comprehension of the mechanisms mediating intracellular APN signaling remains incomplete. We recently demonstrate the antioxidant/antinitrative effects of APN are not dependent on AMPK. Protein kinase A (PKA) has been previously shown to be activated by APN, with uncertain relevance to APN cardiac protection. The current study determined whether the antioxidative/antinitrative effect of APN is mediated by PKA. Administration of APN (2 μg/g) 10 min before reperfusion significantly enhanced cardiac PKA activity, reduced oxidative stress, and decreased infarct size. Knockdown of cardiac PKA expression (PKA-KD) by …

Sevoflurane Induces Cardioprotection Through Reactive Oxygen Species-Mediated Upregulation Of Autophagy In Isolated Guinea Pig Hearts., Mayumi Shiomi, Masami Miyamae, Genzou Takemura, Kazuhiro Kaneda, Yoshitaka Inamura, Anna Onishi, Shizuka Koshinuma, Yoshihiro Momota, Toshiaki Minami, Vincent M. Figueredo

Division of Cardiology Faculty Papers

PURPOSE: Sevoflurane increases reactive oxygen species (ROS), which mediate cardioprotection against myocardial ischemia-reperfusion injury. Emerging evidence suggests that autophagy is involved in cardioprotection. We examined whether reactive oxygen species mediate sevoflurane preconditioning through autophagy.

METHODS: Isolated guinea pigs hearts were subjected to 30 min ischemia followed by 120 min reperfusion (control). Anesthetic preconditioning was elicited with 2 % sevoflurane for 10 min before ischemia (SEVO). The ROS-scavenger, N-(2-mercaptopropionyl) glycine (MPG, 1 mmol/l), was administered starting 30 min before ischemia to sevoflurane-treated (SEVO + MPG) or non-sevoflurane-treated (MPG) hearts. Infarct size was determined by triphenyltetrazolium chloride stain. Tissue samples were obtained …

The Ciliary Protein Cystin Forms A Regulatory Complex With Necdin To Modulate Myc Expression, Maoqing Wu, Chaozhe Yang, Binli Tao, Su Bu, Lisa Guay-Woodford

Pediatrics Faculty Publications

Cystin is a novel cilia-associated protein that is disrupted in the cpk mouse, a well-characterized mouse model of autosomal recessive polycystic kidney disease (ARPKD). Interestingly, overexpression of the Myc gene is evident in animal models of ARPKD and is thought to contribute to the renal cystic phenotype. Using a yeast two-hybrid approach, the growth suppressor protein necdin, known to modulate Myc expression, was found as an interacting partner of cystin. Deletion mapping demonstrated that the C-terminus of cystin and both termini of necdin are required for their mutual interaction. Speculating that these two proteins may function to regulate gene expression, …

Clinically Relevant Intronic Splicing Enhancer Mutation In Myelin Proteolipid Protein Leads To Progressive Microglia And Astrocyte Activation In White And Gray Matter Regions Of The Brain, Adam D. Bachstetter, Scott J. Webster, Linda J. Van Eldik, Franca Cambi

Sanders-Brown Center on Aging Faculty Publications

INTRODUCTION: Mutations in proteolipid protein (PLP), the most abundant myelin protein in the CNS, cause the X-linked dysmyelinating leukodystrophies, Pelizaeus-Merzbacher disease (PMD) and spastic paraplegia type 2 (SPG2). Point mutations, deletion, and duplication of the PLP1 gene cause PMD/SPG2 with varying clinical presentation. Deletion of an intronic splicing enhancer (ISEdel) within intron 3 of the PLP1 gene is associated with a mild form of PMD. Clinical and preclinical studies have indicated that mutations in myelin proteins, including PLP, can induce neuroinflammation, but the temporal and spatial onset of the reactive glia response in a clinically relevant mild form of PMD …