Digital Commons Network^™

Compositions And Methods For Controlling Plant Parasitic Nematodes, Harold N. Trick, Judith L. Roe, Timothy C. Todd, Michael A. Herman

School of Biological Sciences: Faculty Publications

The present invention relates to compositions and methods for controlling nematode infestation of plants. In particular, the present invention provides vectors comprising sequences designed to control nematodes by RNA interference (RNAi) and transgenic plants transformed with Such vectors.

Challenges And Counter Challenges In Hiv/Aids, Qingsheng Li, Charles Wood

Qingsheng Li Publications

Since the beginning of the HIV/AIDS pandemic, there has been significant progress in combating the virus. In addition to identifying HIV,¹ scientists have developed an array of antiretroviral drugs and successfully converted the once deadly disease into a treatable and chronic condition.² Nevertheless, this battle is far from over and HIV still outsmarts humans in multiple ways and poses tremendous challenges. Within this context, the Chinese Medical Journal has timely published this special issue on HIV/AIDS, highlighting the ongoing challenges posed by HIV and potential solutions to these problems. The most daunting challenge is to develop prophylactic measures …

Molecular Basis Of A Novel Adaptation To Hypoxic-Hypercapnia In A Strictly Fossorial Mole, Kevin L. Campbell, Jay F. Storz, Anthony V. Signore, Hideaki Moriyama, Kenneth C. Catania, Alexander P. Payson, Joseph Bonaventura, Jörg Stetefeld, Roy E. Weber

Jay F. Storz Publications

Background: Elevated blood O₂ affinity enhances survival at low O₂ pressures, and is perhaps the best known and most broadly accepted evolutionary adjustment of terrestrial vertebrates to environmental hypoxia. This phenotype arises by increasing the intrinsic O₂ affinity of the hemoglobin (Hb) molecule, by decreasing the intracellular concentration of allosteric effectors (e.g., 2,3-diphosphoglycerate; DPG), or by suppressing the sensitivity of Hb to these physiological cofactors.

Results: Here we report that strictly fossorial eastern moles (Scalopus aquaticus) have evolved a low O₂ affinity, DPG-insensitive Hb - contrary to expectations for a mammalian species that is …

Phenotypic Plasticity And Genetic Adaptation To High-Altitude Hypoxia In Vertebrates, Jay F. Storz, Graham R. Scott, Zachary A. Cheviron

Jay F. Storz Publications

High-altitude environments provide ideal testing grounds for investigations of mechanism and process in physiological adaptation. In vertebrates, much of our understanding of the acclimatization response to high-altitude hypoxia derives from studies of animal species that are native to lowland environments. Such studies can indicate whether phenotypic plasticity will generally facilitate or impede adaptation to high altitude. Here, we review general mechanisms of physiological acclimatization and genetic adaptation to high-altitude hypoxia in birds and mammals. We evaluate whether the acclimatization response to environmental hypoxia can be regarded generally as a mechanism of adaptive phenotypic plasticity, or whether it might sometimes represent …

Genetic Differences In Hemoglobin Function Between Highland And Lowland Deer Mice, Jay F. Storz, Amy M. Runck, Hideaki Moriyama, Roy E. Weber, Angela Fago

Jay F. Storz Publications

In high-altitude vertebrates, adaptive changes in blood–O₂ affinity may be mediated by modifications of hemoglobin (Hb) structure that affect intrinsic O₂ affinity and/or responsiveness to allosteric effectors that modulate Hb–O₂ affinity. This mode of genotypic specialization is considered typical of mammalian species that are high-altitude natives. Here we investigated genetically based differences in Hb–O₂ affinity between highland and lowland populations of the deer mouse (Peromyscus maniculatus), a generalist species that has the broadest altitudinal distribution of any North American mammal. The results of a combined genetic and proteomic analysis revealed that deer mice harbor …

Nebraska Agricultural Water Management Demonstration Network (Nawmdn): Integrating Research And Extension/Outreach, Suat Irmak, Jennifer M. Rees, Gary L. Zoubek, Brandy S. Van Dewalle, William R. Rathje, Rodney Debuhr, Dan Leininger, Darrel D. Siekman, James W. Schneider, Andrew P. Christiansen

Department of Agricultural and Biological Systems Engineering: Faculty Publications

Maximizing the net benefits of irrigated plant production through appropriately designed agricultural water management programs is of growing importance in Nebraska, and other western and Midwestern states, because many areas are involved in management and policy changes to conserve irrigation water. In Nebraska, farmers are being challenged to practice conservation methods and use water resources more efficiently while meeting plant water requirements and maintaining high yields. Another challenge Nebraska experiences in it's approximately 3.5‐million‐ha irrigated lands is limited adoption of newer technologies/tools to help farmers better manage irrigation, conserve water and energy, and increase plant water use efficiency. In 2005, …

Evolutionary Aspects Of Urea Utilization By Fungi, Dhammika H. M. L. P Navarathna, Steven D. Harris, David D. Roberts, Kenneth W. Nickerson

Kenneth Nickerson Papers

The higher fungi exhibit a dichotomy with regard to urea utilization. The hemiascomycetes use urea amidolyase (DUR1,2), whereas all other higher fungi use the nickel-containing urease. Urea amidolyase is an energy-dependent biotincontaining enzyme. It likely arose before the Euascomycete/Hemiascomycete divergence c. 350 million years ago by insertion of an unknown gene into one copy of a duplicated methylcrotonyl CoA carboxylase (MccA). The dichotomy between urease and urea amidolyase coincides precisely with that for the Ni/Co transporter (Nic1p), which is present in the higher fungi that use urease and is absent in those that do not. We suggest that …

Candida Albicans Cellwall Components And Farnesol Stimulate The Expression Of Both Inflammatory And Regulatory Cytokines In The Murine Raw264.7 Macrophage Cell Line, Suman Ghosh, Nina Howe, Katie Volk, Swetha Tati, Kenneth W. Nickerson, Thomas M. Petro

Kenneth Nickerson Papers

Candida albicans causes candidiasis, secretes farnesol, and switches from yeast to hyphae to escape from macrophages after phagocytosis. However, before escape, macrophages may respond to C. albicans’ pathogen-associated molecular patterns (PAMPs) through toll-like receptor 2 (TLR2) and dectin-1 receptors by expressing cytokines involved in adaptive immunity, inflammation, and immune regulation. Therefore, macrophages and the RAW264.7 macrophage line were challenged with C. albicans preparations of live wild-type cells, heat-killed cells, a live mutant defective in hyphae formation, a live mutant producing less farnesol, or an isolate producing farnesoic acid instead of farnesol. Interleukin-6 (IL-6), and IL-1b, IL- 10, and tumor …

No Interaction Between Competition And Herbivory In Limiting Introduced Cirsium Vulgare Rosette Growth And Reproduction, Tomomi  Suwa, Svata M. Louda, F. Leland Russell

Svata M. Louda Publications

Both competition and herbivory have been shown to reduce plant survival, growth, and reproduction. Much less is known about whether competition and herbivory interact in determining plant performance, especially for introduced, weedy plant species in the invaded habitat. We simultaneously evaluated both the main and interactive effects of plant neighbors and insect herbivory on rosette growth and seed reproduction in the year of flowering for Cirsium vulgare (bull thistle, spear thistle), an introduced Eurasian species, in tallgrass prairie in 2 years. Effects of insect herbivory were strong and consistent in both years, causing reduced plant growth and seed production, whereas …

Sirnas Compete With Mirnas For Methylation By Hen1 In Arabidopsis, Bin Yu, Liu Bi, Jixian Zhai, Manu Agarwal, Shengben Li, Qingfa Wu, Shou-Wei Ding, Blake C. Meyers, Hervé Vaucheret, Xuemei Chen

Bin Yu Publications

Plant microRNAs (miRNAs) and small interfering RNAs (siRNAs) bear a 2'-O-methyl group on the 3'-terminal nucleotide. This methyl group is post-synthetically added by the methyltransferase protein HEN1 and protects small RNAs from enzymatic activities that target the 3'-OH. A mutagenesis screen for suppressors of the partial loss-offunction hen1-2 allele in Arabidopsis identified second-site mutations that restore miRNA methylation. These mutations affect two subunits of the DNA-dependent RNA polymerase IV (Pol IV), which is essential for the biogenesis of 24 nt endogenous siRNAs. A mutation in RNA-dependent RNA polymerase 2, another essential gene for the biogenesis of endogenous 24-nt …

Structural Restoration Of Nematodes And Acanthocephalans Fixed In High Percentage Alcohol Using Dess Solution And Rehydration, Soraya Naem, Christopher Pagan, Steven A. Nadler

Harold W. Manter Laboratory of Parasitology: Faculty Publications

Ninety-five percent ethanol is the most widely used field and laboratory preservative for nematodes and other helminth specimens intended for use in molecular systematics. Preservation of nematodes in high-concentration alcohols results in structural dehydration artifacts, including shrinkage and body surface distortions sufficient to obscure features required for morphological identification and analysis, thereby compromising precise morphometrics. However, treating dehydrated nematodes using a solution of DMSO, disodium EDTA, and NaCl, followed by rehydration in water produces marked improvements in specimen shape and surface features, resulting from diffusion of water into the tissues and pseudocoelom as the internal salt concentration is reduced. Following …

Soil-Transmitted Helminthiases: Implications Of Climate Change And Human Behavior, Haylee J. Weaver, John M. Hawdon, Eric P. Hoberg

Harold W. Manter Laboratory of Parasitology: Faculty Publications

Soil-transmitted helminthiases (STHs) collectively cause the highest global burden of parasitic disease after malaria and are most prevalent in the poorest communities, especially in sub-Saharan Africa. Climate change is predicted to alter the physical environment through cumulative impacts of warming and extreme fluctuations in temperature and precipitation, with cascading effects on human health and wellbeing, food security and socioeconomic infrastructure. Understanding how the spectrum of climate change effects will influence STHs is therefore of critical importance to the control of the global burden of human parasitic disease. Realistic progress in the global control of STH in a changing climate requires …

Probing Equilibrium By Nonequilibrium Dynamics: Aging In Co/Cr Superlattices, Tathagata Mukherjee, M. Pleimling, Christian Binek

Christian Binek Publications

Magnetization relaxation is investigated in a structurally ordered magnetic Co/Cr superlattice. Tailored nanoscale periodicity creates mesoscopic spatial magnetic correlations with slow relaxation dynamics when quenching the system into a nonequilibrium state. Magnetization transients are measured after exposing the heterostructure to a magnetic set field for various waiting times. Scaling analysis reveals an asymptotic powerlaw behavior in accordance with a full aging scenario. The temperature dependence of the relaxation exponent shows pronounced anomalies at the equilibrium phase transitions of the antiferromagnetic superstructure and the ferromagnetic to paramagnetic transition of the Co layers. The latter leaves only weak fingerprints in the equilibrium …

Muellerius Capillaris Dominates The Lungworm Community Of Bighorn Sheep At The National Bison Range, Montana, Vanessa O. Ezenwa, Alicia M. Hines, Elizabeth A. Archie, Eric P. Hoberg, Ingrid M. Asmundsson, John T. Hogg

Harold W. Manter Laboratory of Parasitology: Faculty Publications

Lungworm infections are common among bighorn sheep (Ovis canadensis) in North America, and the predominant species reported are Protostrongylus stilesi and P. rushi. The only records of another lungworm species, Muellerius capillaris, infecting bighorns come from South Dakota, USA. At the National Bison Range (NBR), Montana, USA we found that across six sampling periods, 100% of wild bighorn sheep surveyed were passing first-stage dorsal-spined larvae (DSL) which appeared to be consistent with M. capillaris. By contrast, only 39%or fewer sheep were passing Protostrongylus larvae. Using molecular techniques, we positively identified the DSL from the NBR …

A Spirocyclohexyl Nitroxide Amino Acid Spin Label For Pulsed Epr Distance Measurements, Andrzej Rajca, Velavan Kathirvelu, Sandip K. Riy, Maren Pink, Suchada Rajca, Santanu Sarkar, Sandra S. Eaton, Gareth R. Eaton

Andrzej Rajca Publications

Site-directed spin labeling (SDSL) and electron paramagnetic resonance (EPR) spectroscopy offer accurate, sensitive tools for the characterization of structure and function of macromolecules and their assemblies. A new rigid spin label, spirocyclohexyl nitroxide α-amino acid and its N-(9- fluorenylmethoxycarbonyl) (Fmoc) derivative, has been synthesized that exhibit slow enough spin echo dephasing to permit accurate distance measurements by pulse EPR at temperatures up to 125 K in 1:1 water:glycerol and at higher temperatures in matrices with higher glass transition temperatures. Distance measurements in the liquid nitrogen temperature range are less expensive than those that require liquid helium, which will greatly facilitate …

Profess: A Protein Function, Evolution, Structure And Sequence Database, Thomas Triplet, Matthew D. Shortridge, Mark A. Griep, Jaime L. Stark, Robert Powers, Peter Revesz

Robert Powers Publications

The proliferation of biological databases and the easy access enabled by the Internet is having a beneficial impact on biological sciences and transforming the way research is conducted. There are ~1100 molecular biology databases dispersed throughout the Internet. To assist in the functional, structural and evolutionary analysis of the abundant number of novel proteins continually identified from whole-genome sequencing, we introduce the PROFESS (PROtein Function, Evolution, Structure and Sequence) database. Our database is designed to be versatile and expandable and will not confine analysis to a pre-existing set of data relationships. A fundamental component of this approach is the development …

Using Nmr Metabolomics To Investigate Tricarboxylic Acid Cycle-Dependent Signal Transduction In Staphylococcus Epidermidis, Marat R. Sadykov, Bo Zhang, Steven M. Halouska, Jennifer L. Nelson, Lauren W. Kreimer, Yefei Zhu, Robert Powers, Greg A. Somerville

Robert Powers Publications

Staphylococcus epidermidis is a skin-resident bacterium and a major cause of biomaterial-associated infections. The transition from residing on the skin to residing on an implanted biomaterial is accompanied by regulatory changes that facilitate bacterial survival in the new environment. These regulatory changes are dependent upon the ability of bacteria to “sense” environmental changes. In S. epidermidis, disparate environmental signals can affect synthesis of the biofilm matrix polysaccharide intercellular adhesin (PIA). Previously, we demonstrated that PIA biosynthesis is regulated by tricarboxylic acid (TCA) cycle activity. The observations that very different environmental signals result in a common phenotype (i.e. increased PIA …

Solution Structure And Function Of Yndb, An Ahsa1 Protein From Bacillus Subtilis, Jaime L. Stark, Kelly A. Mercier, Geoffrey A. Mueller, Thomas B. Acton, Rong Xiao, Gaetano T. Montelione, Robert Powers

Robert Powers Publications

The solution structure of the Bacillus subtilis protein YndB has been solved using NMR in order to investigate proposed biological functions. The YndB structure exhibits the helix-grip fold, which consists of a β-sheet with two small and one long α-helix, forming a hydrophobic cavity that preferentially binds lipid-like molecules. Sequence and structure comparisons to proteins from eukaryotes, prokaryotes, and archaea suggest that YndB is very similar to the eukaryote protein Aha1, which binds to the middle domain of Hsp90 and induces ATPase activity. Based on these similarities, YndB has been classified as a member of the Activator of Hsp90 ATPase …

New Records Of Bats From The British Virgin Islands, Gary G. Kwiecinski, Jean-Pierre Bacle, Kevel C. Lindsay, Hugh H. Genoways

University of Nebraska State Museum: Mammalogy Papers

As currently understood the bat fauna of the British Virgin Islands consists of five species – Noctilio leporinus, Brachyphylla cavernarum, Artibeus jamaicensis, Tadarida brasiliensis , and Molossus molossus. Our knowledge of distribution of bats in the British Virgin Islands is far more limited than that in the United States Virgin Islands. As part of ongoing research on the bats of the Virgin Islands, recent brief surveying periods in the British Virgin Islands have produced new records for some islands. Also, our researching of existing museum collections has discovered unreported new records. Our new data adds information for five species …

Influence Of Commodity Type, Percentage Of Cracked Kernels, And Wheat Class On Population Growth Of Stored-Product Psocids (Psocoptera: Liposcelidae), Christos G. Athanassiou, George P. Opit, James E. Throne

United States Department of Agriculture-Agricultural Research Service / University of Nebraska-Lincoln: Faculty Publications

Differences in stored-product psocid progeny production as a function of commodity type, percentage of cracked kernels, and wheat class were examined using laboratory bioassays. Population growth of Liposcelis bostrychophila Badonnel, Liposcelis decolor (Pearman), Liposcelis paeta Pearman, and Liposcelis entomophila (Enderlein) (Psocoptera: Liposcelididae) was highest on sorghum Sorghum bicolor (L.) Moench, followed by wheat, Triticum aestivum L., and rice, Oryza sativa L., whereas progeny production was negligible on wheat germ. In a second experiment that did not include L. entomophila, population growth was examined on wheat containing 0, 1, 5, 10, 20, 50, and 100% cracked kernels. Progeny production did …

Current Status And Future Directions Of Precision Aerial Application For Site-Specific Crop Management In The Usa, Yubin Lan, Steven J. Thomson, Yanbo Huang, W. Clint Hoffmann, Huihui Zhang

United States Department of Agriculture-Agricultural Research Service / University of Nebraska-Lincoln: Faculty Publications

The first variable-rate aerial application system was developed about a decade ago in the USA and since then, aerial application has benefitted from these technologies. Many areas of the United States rely on readily available agricultural airplanes or helicopters for pest management, and variable-rate aerial application provides a solution for applying field inputs such as cotton growth regulators, defoliants, and insecticides. In the context of aerial application, variable-rate control can simply mean terminating spray over field areas that do not require inputs, terminating spray near pre-defined buffer areas determined by Global Positioning, or applying multiple rates to meet the variable …

Tuberculosis Vaccine, Mohammed A. Sarhan

Saudi Medical Journal

Tuberculosis (TB) is still one of the leading causes of death from a single infectious agent, killing 1.6 million people each year, mostly in developing countries. The existing vaccines, Bacille Calmette and Guerin (BCG), are efficient in preventing the most severe disseminated forms of disease in children and newborns, but its efficacy against active TB in adults has been challenged by several clinical studies. It is a common opinion that only the development of a new and more effective vaccine against TB would significantly ease the deadly disease. In recent years, looking for a new vaccine or an improved TB …

Efficacy Of Infliximab On The Acute Attack Of Uveitis, Fahdah Alokaily, Sulaiman Alsaleh, Maryseon Al-Balawi, Seham Al-Rashidi

Saudi Medical Journal

Uveitis is one of the major features of behcet9s disease with relapse and remission pattern. Severe presentation can be resistant to the conventional treatment. Steroid has been used in the treatment of acute behcet9s uveitis, although it especially has its own ocular complications when used in large doses. We describe to patients presented with acute uveitis while on treatment, uveitis was controlled by adding anti-tumor necrosis factor (TNF) and Infliximab.

Confirmatory Factor Analysis Of The Child Feeding Questionnaire Among Low-Income African American Families Of Preschool Children, Richard E. Boles, Timothy D. Nelson, Leigh A. Chamberlin, Jessica M. Valenzuela, Susan N. Sherman, Susan L. Johnson, Scott W. Powers

Department of Psychology: Faculty Publications

This study examined the factor structure for three of the Child Feeding Questionnaire (CFQ) subscales, a widely used measure of parental feeding practices, among 296 low-income parents of African American preschool children. Confirmatory factor analysis showed an overall poor fit among CFQ subscales; Restriction, Pressure to Eat, and Concern about Child Weight, (χ2, (df = 87 = 300.249, CFI = 1.00, NNFI = 1.07, RMSEA = .091). Additionally, Cronbach’s Alpha coefficients for 2 of the three subscales were below acceptable recommendations (Restriction = 0.69; Pressure to Eat = 0.58). These results suggest further psychometric clarification is needed to understand commonly …

Corrosion Of Civil War Era Sub Marine Explorer—Part 1, Donald L. Johnson, James D. Carr, Brent M. Wilson, Larry E. Murphy, James P. Delgado

Department of Chemistry: Faculty Publications

The Sub Marine Explorer is one of five submersibles (submarines) constructed prior to 1870 that have survived either in museums or as in situ archaeological sites around the world. Since 1869, the wreck of Explorer has emerged at low tide on the beach of Isla San Telmo, Archipiélago de las Perlas, Panama, located ~75 km southwest of Panama City in the Bay of Panama. In 2001, James Delgado visited the site. Locals described the wreck as a World War II-era Japanese midget submarine. Delgado consulted with Richard Wills, an expert on American Civil War submarines, and confirmed that the well-preserved …

Purification And Characterization Of Cytoplasmic Nadp+- Isocitrate Dehydrogenase, And Amplification Of The Nadp+-Idh Gene From The Wing-Dimorphic Sand Field Cricket, Gryllus Firmus, Anthony J. Zera, Susan Newman, David Berkheim, Christine Black, Lindsay Klug, Erica Crone

School of Biological Sciences: Faculty Publications

Cytoplasmic NADP+-isocitrate dehydrogenase (NADP+-IDH) has been purified and characterized, and its gene sequenced in many animal, plant, and yeast species. However, much less information is available on this enzyme-gene in insects. As a first step in investigating the biochemical and molecular mechanisms by which NADP+-IDH contributes to adaptations for flight vs. reproduction in insects, the enzyme was purified to homogeneity in the wing-dimorphic cricket, Gryllus firmus, characterized, and its corresponding gene sequenced. Using a combination of polyethylene glycol precipitation, Cibacron-Blue affinity chromatography, and hydrophobic interaction chromatography the enzyme was purified 291-fold (7% yield; specific activity = 15.8 μmol NADPH/min/mg …

Seed Availability And Insect Herbivory Limit Recruitment And Adult Density Of Native Tall Thistle, F. Leland Russell, Karen E. Rose, Svata M. Louda

School of Biological Sciences: Faculty Publications

Understanding spatial and temporal variation in factors influencing plant regeneration is critical to predicting plant population growth. We experimentally evaluated seed limitation, insect herbivory, and their interaction in the regeneration and density of tall thistle (Cirsium altissimum) across a topographic ecosystem productivity gradient in tallgrass prairie over two years. On ridges and in valleys, we used a factorial experiment manipulating seed availability and insect herbivory to quantify effects of: seed input on seedling density, insect herbivory on juvenile density, and cumulative impacts of both seed input and herbivory on reproductive adult density. Seed addition increased seedling densities at …

The Polypeptide Syn67 Interacts Physically With Human Holocarboxylase Synthetase, But Is Not A Target For Biotinylation, Yousef I. Hassan, Hideaki Moriyama, Janos Zempleni

School of Biological Sciences: Faculty Publications

Holocarboxylase synthetase (HCS) catalyzes the binding of biotin to lysines in carboxylases and histones in two steps. First, HCS catalyzes the synthesis of biotinyl-5′-AMP; second, the biotinyl moiety is ligated to lysine residues. It has been proposed that step two is fairly promiscuous, and that protein biotinylation may occur in the absence of HCS as long as sufficient exogenous biotinyl-5′- AMP is provided. Here, we identified a novel polypeptide (Syn67) with a basic patch of lysines and arginines. Yeast-two-hybrid assays and limited proteolysis assays revealed that both N- and C-termini of HCS interact with Syn67. A potential target lysine in …

Molecular Determinants Of Hiv-1 Subtype C Coreceptor Transition From R5 To R5x4, Hong Zhang, Damien C. Tully, Tiejun Zhang, Hideaki Moriyama, Jesse Thompson, Charles Wood

School of Biological Sciences: Faculty Publications

The molecular mechanism(s) underlying transition from CCR5 to CXCR4 usage of subtype C viruses remain largely unknown. We previously identified a subtype C HIV-1 infected child whose virus demonstrated CXCR4 usage along with CCR5 upon longitudinal follow-up. Here we delineated the molecular determinants of Env involved in expanded coreceptor usage. Residue changes in three positions of Env V3 domain are critical for the dual-tropic phenotype. These include: substitution of arginine at position 11, MG or LG insertion between positions 13 and 14, and substitution of threonine at the position immediately downstream of the GPGQ crown. Introducing these mutations into V3 …

Transgenic Rat Model Of Neurodegeneration Caused By Mutation In The Tdp Gene, Hongxia Zhou, Cao Huang, Han Chen, Dian Wang, Carlisle P. Landel, Pedro Yuxing Xia, Robert Bowser, Yong-Jian Liu, Xu Gang Xia

Nebraska Center for Biotechnology: Faculty and Staff Publications

TDP-43 proteinopathies have been observed in a wide range of neurodegenerative diseases. Mutations in the gene encoding TDP-43 (i.e., TDP) have been identified in amyotrophic lateral sclerosis (ALS) and in frontotemporal lobe degeneration associated with motor neuron disease. To study the consequences of TDP mutation in an intact system, we created transgenic rats expressing normal human TDP or a mutant form of human TDP with a M337V substitution. Overexpression of mutant, but not normal, TDP caused widespread neurodegeneration that predominantly affected the motor system. TDP mutation reproduced ALS phenotypes in transgenic rats, as seen by progressive degeneration of motor neurons …