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Developing An Operational Definition Of Intellectual Disability For The Purpose Of National Health Surveillance, Alexandra Bonardi, Emily Lauer 2011 University of Massachusetts Medical School

Developing An Operational Definition Of Intellectual Disability For The Purpose Of National Health Surveillance, Alexandra Bonardi, Emily Lauer

Commonwealth Medicine Publications

This report summarizes recommendations that were developed to establish a more consistent approach to operationalizing the case definition for the purpose of public health surveillance among adults with intellectual disability in the United States. The approach included consideration of well established conceptual definitions, such as in the International Classification of Disability, Health, and Function (ICF), and the most recent version of the definition established by the American Association on Intellectual Disability (AAIDD). Additionally, the definitions used in current national and state level major data collection efforts, as well as those used in targeted research studies were considered, with a recognition ...


Assessment Of Olfactory Processing In Parkinson’S Disease Patients, Mary Linton Peters, Jacob Kleinman, Wei Huang, Keith A. Cauley, Paula D. Ravin, Peter Novak, Ali Bourisly, Jean A. King, Julie G. Pilitsis 2011 University of Massachusetts Medical School

Assessment Of Olfactory Processing In Parkinson’S Disease Patients, Mary Linton Peters, Jacob Kleinman, Wei Huang, Keith A. Cauley, Paula D. Ravin, Peter Novak, Ali Bourisly, Jean A. King, Julie G. Pilitsis

Jean A. King

Background: Hyposmia is an early symptom of Parkinson’s Disease (PD) that often predates motor symptoms by years. Hyposmia has been shown to have a more consistent link to idiopathic PD than to other movement disorders. Olfaction has the potential to be used as a biomarker for PD, either through clinical evaluation or imaging. Objectives: This study uses functional magnetic resonance imaging (fMRI) to assess differences in olfaction pathways between anosmic early PD patients and age and gender-matched controls. Methods: 12 PD patients and 12 age- and gender-matched control subjects were recruited from the subject panel of a previous UMMS ...


A Brain-Derived Mecp2 Complex Supports A Role For Mecp2 In Rna Processing, Steven W. Long, Jenny Y. Y. Ooi, Peter M. Yau, Peter L. Jones 2011 University of Illinois at Urbana-Champaign

A Brain-Derived Mecp2 Complex Supports A Role For Mecp2 In Rna Processing, Steven W. Long, Jenny Y. Y. Ooi, Peter M. Yau, Peter L. Jones

Peter Jones Lab Publications

Mutations in MECP2 (methyl-CpG-binding protein 2) are linked to the severe postnatal neurodevelopmental disorder RTT (Rett syndrome). MeCP2 was originally characterized as a transcriptional repressor that preferentially bound methylated DNA; however, recent results indicate MeCP2 is a multifunctional protein. MeCP2 binding is now associated with certain expressed genes and involved in nuclear organization as well, indicating that its gene regulatory function is context-dependent. In addition, MeCP2 is proposed to regulate mRNA splicing and a mouse model for RTT shows aberrant mRNA splicing. To further understand MeCP2 and potential roles in RTT pathogenesis, we have employed a biochemical approach to identify ...


The Role Of Sonic Hedgehog In The Hypothalamus, Solsire E. Zevallos 2011 University of Pennsylvania

The Role Of Sonic Hedgehog In The Hypothalamus, Solsire E. Zevallos

Publicly Accessible Penn Dissertations

ABSTRACT

THE REQUIREMENT OF SONIC HEDGEHOG IN THE HYPOTHALAMUS

Solsire E. Zevallos

Douglas J. Epstein

Sonic hedgehog (Shh) is a morphogen secreted during early development that is required for the formation of the ventral neural tube, including the ventral forebrain. The prechordal source of Shh underlying the forebrain is required early to bifurcate the cerebral hemispheres and eye vesicles as well as to specify a rostroventral forebrain region, the hypothalamus. We hypothesized that Shh expressed later in the hypothalamus may be required for other known functions of Shh, such as patterning within a region, cell proliferation, cell specification, and axon ...


Prevention Of Alzheimer's Disease In High Risk Groups: Statin Therapy In Subjects With Psen1 Mutations Or Heterozygosity For Apolipoprotein E Epsilon 4, Daniel Pollen, Stephen Baker, Douglas Hinerfeld, Joan Swearer, Barbara Evans, James Evans, Richard Caselli, Ekaterina Rogaeva, Peter St. George-Hyslop, Majaz Moonis 2011 University of Massachusetts Medical School

Prevention Of Alzheimer's Disease In High Risk Groups: Statin Therapy In Subjects With Psen1 Mutations Or Heterozygosity For Apolipoprotein E Epsilon 4, Daniel Pollen, Stephen Baker, Douglas Hinerfeld, Joan Swearer, Barbara Evans, James Evans, Richard Caselli, Ekaterina Rogaeva, Peter St. George-Hyslop, Majaz Moonis

Stephen P. Baker

Because cerebrospinal fluid (CSF) abnormalities in presymptomatic subjects with PSEN1 (presenilin 1) mutations may be observed 4 to 12 years prior to the estimated age at onset, it is possible to test putative therapies on the CSF analytes that correlate with neurodegeneration during this presymptomatic window of clinical opportunity. It is also possible to test the same therapy on a comparison group with increased risk status conferred by both hyperlipidemia and heterozygosity for apolipoprotein Eε4. To our knowledge, the only putative therapy thus far tested in such a common design has been statin therapy. The results of these tests show ...


Upregulation Of Reactive Oxygen Species During The Retrovirus Life Cycle And Their Roles In A Mutant Of Moloney Murine Leukemia Virus, Ts1-Mediated Neurodegeneration, SOO JIN KIM 2011 The University of Texas Graduate School of Biomedical Sciences at Houston

Upregulation Of Reactive Oxygen Species During The Retrovirus Life Cycle And Their Roles In A Mutant Of Moloney Murine Leukemia Virus, Ts1-Mediated Neurodegeneration, Soo Jin Kim

UT GSBS Dissertations and Theses (Open Access)

Viral invasion of the central nervous system (CNS) and development of neurological symptoms is a characteristic of many retroviruses. The mechanism by which retrovirus infection causes neurological dysfunction has yet to be fully elucidated. Given the complexity of the retrovirus-mediated neuropathogenesis, studies using small animal models are extremely valuable. Our laboratory has used a mutant moloney murine leukemia retrovirus, ts1-mediated neurodegneration. We hypothesize that astrocytes play an important role in ts1-induced neurodegeneration since they are retroviral reservoirs and supporting cells for neurons. It has been shown that ts1 is able to infect astrocytes in vivo and in ...


Temporal Uncoupling Of The Dna Methylome And Transcriptional Repression During Embryogenesis, Ozren Bogdanovic, Steven W. Long, Simon J. van Heeringen, Arie B. Brinkman, Jose Luis Gomez-Skarmeta, Hendrik G. Stunnenberg, Peter L. Jones, Gert Jan C. Veenstra 2011 Radboud University Nijmegen

Temporal Uncoupling Of The Dna Methylome And Transcriptional Repression During Embryogenesis, Ozren Bogdanovic, Steven W. Long, Simon J. Van Heeringen, Arie B. Brinkman, Jose Luis Gomez-Skarmeta, Hendrik G. Stunnenberg, Peter L. Jones, Gert Jan C. Veenstra

Peter Jones Lab Publications

DNA methylation is a tightly regulated epigenetic mark associated with transcriptional repression. Next-generation sequencing of purified methylated DNA obtained from early Xenopus tropicalis embryos demonstrates that this genome is heavily methylated during blastula and gastrula stages. Although DNA methylation is largely absent from transcriptional start sites marked with histone H3 lysine 4 trimethylation (H3K4me3), we find both promoters and gene bodies of active genes robustly methylated. In contrast, DNA methylation is absent in large H3K27me3 domains, indicating that these two repression pathways have different roles. Comparison with chromatin state maps of human ES cells reveals strong conservation of epigenetic makeup ...


Mechanics Of Living, Squishy Materials, Alan Crosby 2011 University of Massachusetts - Amherst

Mechanics Of Living, Squishy Materials, Alan Crosby

UMass Center for Clinical and Translational Science Research Retreat

Living materials respond to stresses, or forces, surrounding them. If we aim to promote the growth of healthy tissue, such as in the field of tissue engineering, or limit the growth of unwanted tissue, e.g. cancerous tumors, we must understand the stresses that these tissues experience as they grow and live. How do we measure the elastic modulus of a living material? We will discuss a novel measurement technique that we developed, called cavitation rheology, which can quantitatively measure the mechanical environment of soft tissues from sub-cellular to multi-cellular length scales at arbitrary locations. We are particularly interested in ...


Assessment Of Olfactory Processing In Parkinson’S Disease Patients, Mary Linton Peters, Jacob Kleinman, Wei Huang, Keith A. Cauley, Paula D. Ravin, Peter Novak, Ali Bourisly, Jean A. King, Julie G. Pilitsis 2011 University of Massachusetts Medical School

Assessment Of Olfactory Processing In Parkinson’S Disease Patients, Mary Linton Peters, Jacob Kleinman, Wei Huang, Keith A. Cauley, Paula D. Ravin, Peter Novak, Ali Bourisly, Jean A. King, Julie G. Pilitsis

Senior Scholars Program

Background: Hyposmia is an early symptom of Parkinson’s Disease (PD) that often predates motor symptoms by years. Hyposmia has been shown to have a more consistent link to idiopathic PD than to other movement disorders. Olfaction has the potential to be used as a biomarker for PD, either through clinical evaluation or imaging.

Objectives: This study uses functional magnetic resonance imaging (fMRI) to assess differences in olfaction pathways between anosmic early PD patients and age and gender-matched controls.

Methods: 12 PD patients and 12 age- and gender-matched control subjects were recruited from the subject panel of a previous UMMS ...


Characterization Of Amino Acid Residues Integral To Neuronal Binding Of Amyloid Beta Protein In Alzheimer’S Disease, Nicole C. Olson 2011 California Polytechnic State University - San Luis Obispo

Characterization Of Amino Acid Residues Integral To Neuronal Binding Of Amyloid Beta Protein In Alzheimer’S Disease, Nicole C. Olson

Chemistry and Biochemistry

Purpose: Alzheimer’s Disease is a neurodegenerative disease resulting from over-production and neuronal accumulation of amyloid-beta proteins (Aβ40/Aβ42). The glycine residue at position 33 and histidine residues at positions 13 and 14 are involved with binding and internalization of these proteins, actions potentially inhibited by substituting or sterically hindering these residues with an antibody specific to positions 2-11 (IgG-4.1). Rat pheochromocytoma (PC12) cells differentiated with nerve growth factor were used as a neuronal model to determine whether substitution and/or antibody block amyloid-beta’s neuronal interactions.

Methods: PC12 cells were incubated with fluorescein-labeled-amyloid-beta-40 (F-Aβ40) or substituted F-Aβ40 derivatives ...


Can We Stop The Stroke Epidemic In Pakistan?, Ismail A. Khatri, Mohammad Wasay 2011 Shifa International Hospital

Can We Stop The Stroke Epidemic In Pakistan?, Ismail A. Khatri, Mohammad Wasay

Department of Medicine

No abstract provided.


Establishment Of Clonal Myogenic Cell Lines From Severely Affected Dystrophic Muscles - Cdk4 Maintains The Myogenic Population, Guido Stadler, Jennifer Cj Chen, Kathryn Wagner, Jerome D. Robin, Jerry W. Shay, Charles P. Emerson, Jr., Woodring E. Wright 2011 UT Southwestern Medical Center at Dallas

Establishment Of Clonal Myogenic Cell Lines From Severely Affected Dystrophic Muscles - Cdk4 Maintains The Myogenic Population, Guido Stadler, Jennifer Cj Chen, Kathryn Wagner, Jerome D. Robin, Jerry W. Shay, Charles P. Emerson, Jr., Woodring E. Wright

Wellstone Center for FSHD Publications and Presentations

BACKGROUND: A hallmark of muscular dystrophies is the replacement of muscle by connective tissue. Muscle biopsies from patients severely affected with facioscapulohumeral muscular dystrophy (FSHD) may contain few myogenic cells. Because the chromosomal contraction at 4q35 linked to FSHD is thought to cause a defect within myogenic cells, it is important to study this particular cell type, rather than the fibroblasts and adipocytes of the endomysial fibrosis, to understand the mechanism leading to myopathy.

RESULTS: We present a protocol to establish clonal myogenic cell lines from even severely dystrophic muscle that has been replaced mostly by fat, using overexpression of ...


Does Inorganic Mercury Play A Role In Alzheimer's Disease? A Systematic Review And An Integrated Molecular Mechanism, Joachim Mutter, Annika Curth, Johannes Naumann, Richard Deth, Harald Walach 2011 Department of Environmental and Integrative Medicine - Constance Germany

Does Inorganic Mercury Play A Role In Alzheimer's Disease? A Systematic Review And An Integrated Molecular Mechanism, Joachim Mutter, Annika Curth, Johannes Naumann, Richard Deth, Harald Walach

Richard Deth

Mercury is one of the most toxic substances known to humans. It has been introduced into the human environment and has also been widely used in medicine. Since circumstantial evidence exists that the pathology of Alzheimer’s disease (AD) might be in part caused or exacerbated by inorganic mercury, we conducted a systematic review using a comprehensive search strategy. Studies were screened according to a predefined protocol. Two reviewers extracted relevant data independent of each other. One thousand and forty one references were scrutinized, and 106 studies fulfilled the inclusion criteria. Most studies were case control or comparative cohort studies ...


Delayed Sudden Coma Due To Artery Of Percheron Infarction, Lucia Rivera Lara, Nils Henninger 2011 University of Massachusetts Medical School

Delayed Sudden Coma Due To Artery Of Percheron Infarction, Lucia Rivera Lara, Nils Henninger

Neurology Publications and Presentations

A 52-year-old man was noted to display “unusual behavior” with transient agitation and blurry vision after otherwise uneventful diagnostic cardiac catheterization. Several hours after same-day discharge from the hospital, he suddenly became comatose, requiring intubation and admission to the intensive care unit. Two days later, he regained consciousness and was noted to have vertical gaze palsy and dysarthria without other neurologic deficits. Magnetic resonance imaging demonstrated bilateral acute medial thalamic ischemic strokes. Magnetic resonance angiography did not display extracranial or intracranial arterial stenosis (not shown). At 3 months' follow-up, he had only mild residual dysarthria.


Microrna-Regulated, Systemically Delivered Raav9: A Step Closer To Cns-Restricted Transgene Expression, Jun Xie, Qing Xie, Hongwei Zhang, Stefan L. Ameres, Jui-Hung Hung, Qin Su, Ran He, Xin Mu, Seemin Seher Ahmed, Soyeon Park, Hiroki Kato, Chengjian Li, Christian Mueller, Craig C. Mello, Zhiping Weng, Terence R. Flotte, Phillip D. Zamore, Guangping Gao 2011 University of Massachusetts Medical School

Microrna-Regulated, Systemically Delivered Raav9: A Step Closer To Cns-Restricted Transgene Expression, Jun Xie, Qing Xie, Hongwei Zhang, Stefan L. Ameres, Jui-Hung Hung, Qin Su, Ran He, Xin Mu, Seemin Seher Ahmed, Soyeon Park, Hiroki Kato, Chengjian Li, Christian Mueller, Craig C. Mello, Zhiping Weng, Terence R. Flotte, Phillip D. Zamore, Guangping Gao

Open Access Articles

Recombinant adeno-associated viruses (rAAVs) that can cross the blood-brain-barrier and achieve efficient and stable transvascular gene transfer to the central nervous system (CNS) hold significant promise for treating CNS disorders. However, following intravascular delivery, these vectors also target liver, heart, skeletal muscle, and other tissues, which may cause untoward effects. To circumvent this, we used tissue-specific, endogenous microRNAs (miRNAs) to repress rAAV expression outside the CNS, by engineering perfectly complementary miRNA-binding sites into the rAAV9 genome. This approach allowed simultaneous multi-tissue regulation and CNS-directed stable transgene expression without detectably perturbing the endogenous miRNA pathway. Regulation of rAAV expression by miRNA ...


Phytic Acid Protects Against 6-Hydroxydopamine-Induced Dopaminergic Neuron Apoptosis In Normal And Iron Excess Conditions In A Cell Culturemodel, Qi Xu, Anumantha G. Kanthasamy, Manju B. Reddy 2011 Shanghai University of Traditional Chinese Medicine and Pharmacology

Phytic Acid Protects Against 6-Hydroxydopamine-Induced Dopaminergic Neuron Apoptosis In Normal And Iron Excess Conditions In A Cell Culturemodel, Qi Xu, Anumantha G. Kanthasamy, Manju B. Reddy

Food Science and Human Nutrition Publications

Iron may play an important role in Parkinson’s disease (PD) since it can induce oxidative stress-dependent neurodegeneration. The objective of this study was to determine whether the iron chelator, phytic acid (IP6) can protect against 6-hydroxydopamine- (6-OHDA-) induced apoptosis in immortalized rat mesencephalic dopaminergic cells under normal and iron-excess conditions. Caspase-3 activity was increased about 6-fold after 6-OHDA treatment (compared to control; P < .001) and 30 μmol/L IP6 pretreatment decreased it by 38% (P < .05). Similarly, a 63% protection (P < .001) against 6-OHDA induced DNA fragmentation was observed with IP6 pretreatment. Under iron-excess condition, a 6-fold increase in caspase-3 activity (P < .001) and a 42% increase in DNA fragmentation (P < .05) with 6-OHDA treatment were decreased by 41% (P < .01) and 27% (P < .05), respectively, with 30 μmol/L IP6. Together, our data suggest that IP6 protects against 6-OHDA-induced cell apoptosis in both normal and iron-excess conditions, and IP6 may offer neuroprotection in PD.


Is L-Carnitine An Effective Treatment To Improve The Quality Of Life For Patients With Rett Syndrome?, Bernadette Mason 2011 Philadelphia College of Osteopathic Medicine

Is L-Carnitine An Effective Treatment To Improve The Quality Of Life For Patients With Rett Syndrome?, Bernadette Mason

PCOM Physician Assistant Studies Student Scholarship

Objective: To determine “Is L-carnitine an effective treatment to improve the quality of life for patients with Rett Syndrome?”


Alzheimer's Disease Association With Chlamydia Pneumoniae, Andrew Short 2011 Philadelphia College of Osteopathic Medicine

Alzheimer's Disease Association With Chlamydia Pneumoniae, Andrew Short

PCOM Biomedical Studies Student Scholarship

Exogenous bacteria, such as Chlamydia pneumoniae, may be a cause of inflammation that contributes to the progression of Alzheimer's disease (AD). Inflammation has been previously established as a contributor to AD progression. Balin et al. first reported C. pneumoniae in the brains of post-mortem late onset AD patients. PCR analysis showed that 17/19 AD patients were positive for the organism in areas of the brain with typical AD-related neuropathology while 18/19 control patients were PCR-negative. Gerard et al. determined, using real-time PCR, that C. pneumoniae-infected cells colocalize with both neuritic senile plaques and neurofibrillary tangles, the defining ...


Analysis Of Chlamydia Pneumoniae And Ad-Like Pathology In The Brains Of Balb/C Mice Following Direct Intra-Cranial Infection, Jessica Rachel Barton, Christine J. Hammond, Amy L. Brady, Denah M. Appelt, Brian J. Balin, Christopher Scott Little 2011 Philadelphia College of Osteopathic Medicine

Analysis Of Chlamydia Pneumoniae And Ad-Like Pathology In The Brains Of Balb/C Mice Following Direct Intra-Cranial Infection, Jessica Rachel Barton, Christine J. Hammond, Amy L. Brady, Denah M. Appelt, Brian J. Balin, Christopher Scott Little

Scholarly Posters

Alzheimer’s disease (AD) is an age-related progressive neurodegenerative disorder and the most common form of dementia. The pathology in the central nervous system (CNS) impairs memory and cognition, hindering the capabilities and the quality of life of the individual. This project continues studying the role of infection and Alzheimer’s disease, as previous studies in this laboratory have done, and contributes to the overall understanding of the possible causes of this disease. In this study, BALB/c mice were infected, via direct intracranial injection, with a respiratory isolate (AR-39) of Chlamydia pneumoniae. Their brains were analyzed at 7 and ...


Infection Of Neuronal Cells By Chlamydia Pneumoniae And Herpes Simplex Virus Type 1 Alters Expression Of Genes Associated With Alzheimer’S Disease, Morgan M. Devins, Fiora D. Zoga, Brian J. Balin, Denah M. Appelt, Susan T. Hingley 2011 Phila College of Osteopathic Medicine

Infection Of Neuronal Cells By Chlamydia Pneumoniae And Herpes Simplex Virus Type 1 Alters Expression Of Genes Associated With Alzheimer’S Disease, Morgan M. Devins, Fiora D. Zoga, Brian J. Balin, Denah M. Appelt, Susan T. Hingley

Scholarly Posters

Several studies have suggested an infectious etiology for Alzheimer’s disease (AD). We have been investigating a potential role for both Chlamydia pneumoniae and Herpes simplex virus type 1 (HSV1) in the initiation of sporadic late-onset AD. Our current study focuses on investigation of gene expression using Alzheimer-specific Real-Time PCR microarrays on RNA derived from SKNMC human neuronal cells infected with C. pneumoniae and/or HSV1. There are distinct differences in the patterns of gene regulation by the two pathogens. For example, C. pneumoniae induces expression of genes involved in amyloid production and processing, such as β-amyloid precursor protein (APP ...


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