NDLI: Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.

Please wait, while we are loading the content...

Year: 2016, Volume: 113

Year: 2015, Volume: 112

Year: 2014, Volume: 111

Year: 2013, Volume: 110

Year: 2012, Volume: 109

Year: 2011, Volume: 109

Year: 2011, Volume: 108

Year: 2010, Volume: 107

Year: 2009, Volume: 106

Year: 2009, Volume: 94

Year: 2008, Volume: 105

Year: 2007, Volume: 104

Year: 2006, Volume: 103

Year: 2005, Volume: 103

Year: 2005, Volume: 102

Year: 2004, Volume: 101

Year: 2003, Volume: 100

Year: 2002, Volume: 99

Year: 2001, Volume: 98

Year: 2000, Volume: 97

Year: 1999, Volume: 96

Year: 1998, Volume: 95

Year: 1997, Volume: 94

Year: 1996, Volume: 93

Year: 1996, Volume: 93, Issue: 26

Year: 1996, Volume: 93, Issue: 25

Year: 1996, Volume: 93, Issue: 24

Year: 1996, Volume: 93, Issue: 23

Year: 1996, Volume: 93, Issue: 22

Year: 1996, Volume: 93, Issue: 21

Year: 1996, Volume: 93, Issue: 20

Year: 1996, Volume: 93, Issue: 19

Year: 1996, Volume: 93, Issue: 18

Year: 1996, Volume: 93, Issue: 17

Year: 1996, Volume: 93, Issue: 16

Year: 1996, Volume: 93, Issue: 15

Year: 1996, Volume: 93, Issue: 14

Year: 1996, Volume: 93, Issue: 13

Year: 1996, Volume: 93, Issue: 12

Year: 1996, Volume: 93, Issue: 11

Year: 1996, Volume: 93, Issue: 10

Year: 1996, Volume: 93, Issue: 9

Year: 1996, Volume: 93, Issue: 8

Year: 1996, Volume: 93, Issue: 7

Barriers to protein folding: formation of buried polar interactions is a slow step in acquisition of structure.

Spectroscopic recognition of guanine dimeric hairpin quadruplexes by a carbocyanine dye.

Potentiation of proton transfer function by electrostatic interactions in photosynthetic reaction centers from Rhodobacter sphaeroides: First results from site-directed mutation of the H subunit.

Expression of the CD36 homolog (FAT) in fibroblast cells: effects on fatty acid transport.

Swiveling-domain mechanism for enzymatic phosphotransfer between remote reaction sites.

Isolation of a Pax-6 homolog from the ribbonworm Lineus sanguineus.

Activity of primate inferotemporal neurons related to a sought target in pair-association task.

The human CAS protein which is homologous to the CSE1 yeast chromosome segregation gene product is associated with microtubules and mitotic spindle.

C factor, a cell-surface-associated intercellular signaling protein, stimulates the cytoplasmic Frz signal transduction system in Myxococcus xanthus.

DNA binding specificity of two homeodomain proteins in vitro and in Drosophila embryos.

Human cooperation in the simultaneous and the alternating Prisoner's Dilemma: Pavlov versus Generous Tit-for-Tat.

Hypothesis: "Rogue cell"-type chromosomal damage in lymphocytes is associated with infection with the JC human polyoma virus and has implications for oncopenesis.

Immunohistochemical detection of 4-hydroxynonenal protein adducts in Parkinson disease.

Eradication of human hepatic and pulmonary melanoma metastases in SCID mice by antibody-interleukin 2 fusion proteins.

The genomic and physical organization of Ty1-copia-like sequences as a component of large genomes in Pinus elliottii var. elliottii and other gymnosperms.

Increased mRNA levels for components of the lysosomal, Ca2+-activated, and ATP-ubiquitin-dependent proteolytic pathways in skeletal muscle from head trauma patients.

Regulation of mitochondrial pyruvate dehydrogenase activity by tau protein kinase I/glycogen synthase kinase 3beta in brain.

Heterogeneity of primer extension products in asymmetric PCR is due both to cleavage by a structure-specific exo/endonuclease activity of DNA polymerases and to premature stops.

A mammalian adaptor protein with conserved Src homology 2 and phosphotyrosine-binding domains is related to Shc and is specifically expressed in the brain.

Structural basis for specificity and potency of a flavonoid inhibitor of human CDK2, a cell cycle kinase.

Pentameric subunit stoichiometry of a neuronal glutamate receptor.

Inositol 1,4,5-trisphosphate receptor subtype 3 in pancreatic islet cell secretory granules revisited.

Batimastat, a potent matrix mealloproteinase inhibitor, exhibits an unexpected mode of binding.

Protein synthesis editing by a DNA aptamer.

Occurrence of poly(alpha2,8-deaminoneuraminic acid) in mammalian tissues: widespread and developmentally regulated but highly selective expression on glycoproteins.

Environmental services of biodiversity.

Interface between culturally based preferences and genetic preferences: female mate choice in Poecilia reticulata.

Activation of c-Jun N-terminal kinase in bacterial lipopolysaccharide-stimulated macrophages.

Unexpected beta2-microglobulin sequence diversity in individual rainbow trout.

Apoptosis of hemopoietic cells by the human granulocyte-macrophage colony-stimulating factor mutant E21R.

A stationary-phase stress-response sigma factor from Mycobacterium tuberculosis.

Regulation of meiotic chromatin loop size by chromosomal position.

Transposon tools for recombinant DNA manipulation: characterization of transcriptional regulators from yeast, Xenopus, and mouse.

An inhalational anesthetic binding domain in the nicotinic acetylcholine receptor.

Sequence specificity of triplex DNA formation: Analysis by a combinatorial approach, restriction endonuclease protection selection and amplification.

Association of the Est1 protein with telomerase activity in yeast.

The single Cys2-His2 zinc finger domain of the GAGA protein flanked by basic residues is sufficient for high-affinity specific DNA binding.

G protein subunits and the stimulation of phospholipase C by Gs-and Gi-coupled receptors: Lack of receptor selectivity of Galpha(16) and evidence for a synergic interaction between Gbeta gamma and the alpha subunit of a receptor activated G protein.

The role of NF-kappaB in the angiogenic response of coronary microvessel endothelial cells.

The 5' coding region of Paramecium surface antigen genes controls mutually exclusive transcription.

BEN/SC1/DM-GRASP, a homophilic adhesion molecule, is required for in vitro myeloid colony formation by avian hemopoietic progenitors.

cDNA cloning and gene mapping of a candidate human cell cycle checkpoint protein.

Mutants in the Exo I motif of Escherichia coli dnaQ: defective proofreading and inviability due to error catastrophe.

Reduced sympathetic innervation after alteration of target cell neurotransmitter phenotype in transgenic mice.

Oxygen supply and oxygen-dependent gene expression in differentiating embryonic stem cells.

Accelerated evolution and Muller's rachet in endosymbiotic bacteria.

CpG motifs present in bacteria DNA rapidly induce lymphocytes to secrete interleukin 6, interleukin 12, and interferon gamma.

Aberrant platelet-derived growth factor alpha-receptor transcript as a diagnostic marker for early human germ cell tumors of the adult testis.

Expression of a protective gene-prolongs survival of T cells in human immunodeficiency virus-infected patients.

On the belief that arthritis pain is related to the weather.

Interleukin (IL) 15/IL-T production by the adult T-cell leukemia cell line HuT-102 is associated with a human T-cell lymphotrophic virus type I region /IL-15 fusion message that lacks many upstream AUGs that normally attenuates IL-15 mRNA translation.

Activation of CD4+ T lymphocytes form interleukin 2-deficient mice by costimulatory B7 molecules.

Eradication of established intracranial rat gliomas by transforming growth factor beta antisense gene therapy.

Recombinant human uteroglobin suppresses cellular invasiveness via a novel class of high-affinity cell surface binding site.

In vivo selection of basic region-leucine zipper proteins with altered DNA-binding specificities.

Imaging of single molecule diffusion.

Chronic estrogen-induced cervical and vaginal squamous carcinogenesis in human papillomavirus type 16 transgenic mice.

Amphibian transcription factor IIIA proteins contain a sequence element functionally equivalent to the nuclear export signal of human immunodeficiency virus type 1 Rev.

Mutagenesis by third-strand-directed psoralen adducts in repair-deficient human cells: high frequency and altered spectrum in a xeroderma pigmentosum variant.

High copy number I-Ab transgenes induce production of IgE through an interluekin 4-dependent mechanism.

Bombesin antagonist prevents CO2 laser-induced promotion of oral cancer.

Mammalian ubiquitin-conjugating enzyme Ubc9 interacts with Rad51 recombination protein and localizes in synaptonemal complexes.

Identification of a phospholipase C beta2 region that interacts with Gbeta-gamma.

Formation of chimeric DNA primer extension products by template switching onto an annealed downstream oligonucleotide.

Intradimerically tethered DNA topoisomerase II is catalytically active in DNA transport.

Bidirectional uncoating of the genomic RNA of a helical virus.

Direct measurement of salt-bridge solvation energies using a peptide model system: implications for protein stability.

Thermal unfolding of human high-density apolipoprotein A-1: implications for a lipid-free molten globular state.

Enhanced pathologic properties of Dutch-type mutant amyloid beta-protein.

Comparative analysis of ribonuclease P RNA using gene sequences from natural microbial populations reveals tertiary structural elements.

Isolation of Drosophila cyclin D, a protein expressed in the morphogenetic furrow before entry into S phase.

Dated co-occurrence of Homo erectus and Gigantopithecus from Tham Khuyen Cave, Vietnam.

Social evolution in a new environment: the case of introduced fire ants.

Imprinting of the gene encoding a human cyclin-dependent kinase inhibitor, p57KIP2, on chromosome 11p15.

Intravenous injection of soluble antigen induces thymic and peripheral T-cells apoptosis.

Major histocompatibility complex class I molecule serves as a ligand for presentation of the superantigen staphylococcal enterotoxin B to T cells.

Chronic stress alters the immune response to influenza virus vaccine in older adults.

The genetics of pain and pain inhibition.

Successful expression of human factor IX following repeat administration of adenoviral vector in mice.

In vitro synthesis of oncogenic human papillomaviruses requires episomal genomes for differentiation-dependent late expression.

Protein-tyrosine phosphatase activity regulates osteoclast formation and function: inhibition by alendronate.

Pre-mRNA splicing in plants: characterization of Ser/Arg splicing factors.

Relative effects of female fecundity and male mating success on fertility selection in Drosophila pseudoobscura.

Evolution of GABAergic circuitry in the mammalian medial geniculate body.

Repression of a matrix metalloprotease gene by E1A correlates with its ability to bind to cell type-specific transcription factor AP-2.

RecA protein stimulates homologous recombination in plants.

Crystal structures of the trimeric human immunodeficiency virus type 1 matrix protein: implications for membrane association and assembly.

Identification and characterization of a RING zinc finger gene (C-RZF) expressed in chicken embryo cells.

Developmental abnormalities in cultured mouse embryos deprived of retinoic by inhibition of yolk-sac retinol binding protein synthesis.

Leukemia inhibitory factor (LIF) and LIF receptor expression in human endometrium suggests a potential autocrine/paracrine function in regulating embryo implantation.

Circuit-specific alterations of N-methyl-D-aspartate receptor subunit 1 in the dentate gyrus of aged monkeys.

Inhibition of acute in vivo human immunodeficiency virus infection by human interleukin 10 treatment of SCID mice implanted with human fetal thymus and liver.

An integrated map of the genome of the tubercle bacillus, Mycobacterium tuberculosis H37Rv, and comparison with Mycobacterium leprae.

The open reading frame of bamboo mosaic potexvirus satellite RNA is not essential for its replication and can be replaced with a bacterial gene.

Conformations and folding of lysozyme ions in vacuo.

A high-resolution physical map of human chromosome 11.

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.

Year: 1996, Volume: 93, Issue: 6

Year: 1996, Volume: 93, Issue: 5

Year: 1996, Volume: 93, Issue: 4

Year: 1996, Volume: 93, Issue: 3

Year: 1996, Volume: 93, Issue: 2

Year: 1996, Volume: 93, Issue: 1

Year: 1995, Volume: 92

Year: 1994, Volume: 91

Year: 1993, Volume: 90

Year: 1992, Volume: 89

Year: 1991, Volume: 88

Year: 1990, Volume: 87

Year: 1989, Volume: 86

Year: 1988, Volume: 85

Year: 1987, Volume: 84

Year: 1986, Volume: 83

Year: 1985, Volume: 82

Year: 1984, Volume: 81

Year: 1983, Volume: 80

Year: 1982, Volume: 79

Year: 1981, Volume: 78

Year: 1980, Volume: 77

Year: 1979, Volume: 76

Year: 1978, Volume: 75

Year: 1977, Volume: 74

Year: 1976, Volume: 73

Year: 1975, Volume: 72

Year: 1974, Volume: 71

Year: 1973, Volume: 70

Year: 1972, Volume: 69

Year: 1971, Volume: 68

Year: 1970, Volume: 67

Year: 1970, Volume: 66

Year: 1970, Volume: 65

Year: 1969, Volume: 64

Year: 1969, Volume: 63

Year: 1969, Volume: 62

Year: 1968, Volume: 61

Year: 1968, Volume: 60

Year: 1968, Volume: 59

Year: 1967, Volume: 58

Year: 1967, Volume: 57

Year: 1966, Volume: 56

Year: 1966, Volume: 55

Year: 1965, Volume: 54

Year: 1965, Volume: 53

Year: 1964, Volume: 52

Year: 1964, Volume: 51

Year: 1963, Volume: 50

Year: 1963, Volume: 49

Year: 1962, Volume: 48

Year: 1961, Volume: 47

Year: 1960, Volume: 46

Year: 1959, Volume: 45

Year: 1958, Volume: 44

Year: 1957, Volume: 43

Year: 1956, Volume: 42

Year: 1955, Volume: 41

Year: 1954, Volume: 40

Year: 1953, Volume: 39

Year: 1952, Volume: 38

Year: 1951, Volume: 37

Year: 1950, Volume: 36

Year: 1949, Volume: 35

Year: 1948, Volume: 34

Year: 1947, Volume: 33

Year: 1946, Volume: 32

Year: 1945, Volume: 31

Year: 1944, Volume: 30

Year: 1943, Volume: 29

Year: 1942, Volume: 28

Year: 1941, Volume: 27

Year: 1940, Volume: 26

Year: 1939, Volume: 25

Year: 1938, Volume: 24

Year: 1937, Volume: 23

Year: 1936, Volume: 22

Year: 1935, Volume: 21

Year: 1934, Volume: 20

Year: 1933, Volume: 19

Year: 1932, Volume: 18

Year: 1931, Volume: 17

Year: 1930, Volume: 16

Year: 1929, Volume: 15

Year: 1928, Volume: 14

Year: 1927, Volume: 13

Year: 1926, Volume: 12

Year: 1925, Volume: 11

Year: 1924, Volume: 10

Year: 1923, Volume: 9

Year: 1922, Volume: 8

Year: 1921, Volume: 7

Year: 1920, Volume: 6

Year: 1919, Volume: 5

Year: 1918, Volume: 4

Year: 1917, Volume: 3

Year: 1916, Volume: 2

Year: 1915, Volume: 1

Transgenic mice carrying a human mutant superoxide dismutase transgene develop neuronal cytoskeletal pathology resembling human amyotrophic lateral sclerosis lesions.

Content Provider	PubMed Central
Author	Tu, P. H. Raju, P. Robinson, K. A. Gurney, M. E. Trojanowski, J. Q. Lee, V. M.
Abstract	Mutations in the human Cu,Zn superoxide dismutase gene (SOD1) are found in 20% of kindreds with familial amyotrophic lateral sclerosis. Transgenic mice (line G1H) expressing a human SOD1 containing a mutation of Gly-93 --> Ala (G93A) develop a motor neuron disease similar to familial amyotrophic lateral sclerosis, but transgenic mice (line N1029) expressing a wild-type human SOD1 transgene do not. Because neurofilament (NF)-rich inclusions in spinal motor neurons are characteristic of amyotrophic lateral sclerosis, we asked whether mutant G1H and/or N1029 mice develop similar NF lesions. NF inclusions (i.e., spheroids, Lewy body-like inclusions) were first detected in spinal cord motor neurons of the G1H mice at 82 days of age about the time these mice first showed clinical evidence of disease. Other neuronal intermediate filament proteins (alpha-internexin, peripherin) also accumulated in these spheroids. The onset of accumulations of ubiquitin immunoreactivity in the G1H mice paralleled the emergence of vacuoles and NF-rich spheroids in neurons, but they did not colocalize exclusively with spheroids. In contrast, NF inclusions were not seen in the N1029 mice until they were 132 days old, and ubiquitin immunoreactivity was not increased in the N1029 mice even at 199 days of age. Astrocytosis in spinal cord was associated with a marked increase in glial fibrillary acidic protein immunoreactivity in the G1H mice, but not in the N1029 mice. Finally, comparative studies revealed a striking similarity between the cytoskeletal pathology in the G1H transgenic mice and in patients with amyotrophic lateral sclerosis. These findings link a specific SOD1 mutation with alterations in the neuronal cytoskeleton of patients with amyotrophic lateral sclerosis. Thus, neuronal cytoskeletal abnormalities may be implicated in the pathogenesis of human familial amyotrophic lateral sclerosis.
Starting Page	3155
File Format	PDF
ISSN	10916490
e-ISSN	10916490
Journal	Proceedings of the National Academy of Sciences of the United States of America
Issue Number	7
Volume Number	93
Language	English
Publisher Date	1996-04-02
Access Restriction	Open
Subject Keyword	Research in Higher Education
Content Type	Text
Resource Type	Article
Subject	Multidisciplinary

Central Library (ISO-9001:2015 Certified)
Indian Institute of Technology Kharagpur
Kharagpur, West Bengal, India | PIN - 721302

See location in the Map
03222 282435
Mail: support@ndl.gov.in

Sl.	Authority	Responsibilities	Communication Details
1	Ministry of Education (GoI), Department of Higher Education	Sanctioning Authority	https://www.education.gov.in/ict-initiatives
2	Indian Institute of Technology Kharagpur	Host Institute of the Project: The host institute of the project is responsible for providing infrastructure support and hosting the project	https://www.iitkgp.ac.in
3	National Digital Library of India Office, Indian Institute of Technology Kharagpur	The administrative and infrastructural headquarters of the project	Dr. B. Sutradhar bsutra@ndl.gov.in
4	Project PI / Joint PI	Principal Investigator and Joint Principal Investigators of the project	Dr. B. Sutradhar bsutra@ndl.gov.in Prof. Saswat Chakrabarti will be added soon
5	Website/Portal (Helpdesk)	Queries regarding NDLI and its services	support@ndl.gov.in
6	Contents and Copyright Issues	Queries related to content curation and copyright issues	content@ndl.gov.in
7	National Digital Library of India Club (NDLI Club)	Queries related to NDLI Club formation, support, user awareness program, seminar/symposium, collaboration, social media, promotion, and outreach	clubsupport@ndl.gov.in
8	Digital Preservation Centre (DPC)	Assistance with digitizing and archiving copyright-free printed books	dpc@ndl.gov.in
9	IDR Setup or Support	Queries related to establishment and support of Institutional Digital Repository (IDR) and IDR workshops	idr@ndl.gov.in