Detmer, Scott A., Velde, Christine Vande, Cleveland, Don W., Chan, David C.
Charcot–Marie–Tooth (CMT) disease type 2A is a progressive, neurodegenerative disorder affecting long peripheral motor and sensory nerves. The most common clinical sign is weakness in the lower...
Dhib-Jalbut, Suhayl, Arnold, Douglas L., Cleveland, Don W., Fisher, Marc, Friedlander, Robert M., Mouradian, M. Maral, ...
Multiple sclerosis is considered a disease of myelin destruction; Parkinson's disease (PD), one of dopaminergic neuron depletion; ALS, a disease of motor neuron death; and Alzheimer's, a disease of...
Raoul, Cédric, Estévez, Alvaro G, Nishimune, Hiroshi, Cleveland, Don W, DeLapeyrière, Odile, Henderson, Christopher E, ...
Death pathways restricted to specific neuronal classes could potentially allow for precise control of developmental neuronal death and also underlie the selectivity of neuronal loss in...
Katz, David A., Theodorakis, Nicholas G., Cleveland, Don W., Lindsten, Tullia
The 3′-untranslated regions of many labile transcripts contain AU-rich sequences that serve as cis determinants of mRNA stability and translatlonal efficiency. Using a photocrossllnklng technique,...
Sisodia, Sangram S., Cleveland, Don W., Sollner-Webb, Barbara
SI nucleate napping it commonly used to analyze transcription and processing of unlabellod RNAs. However, the SI protoool that appears best suited to demonstrate splioing of a particular RNA (using...
Borisy, Gary G, Cleveland, Don W, Murphy, Douglas B
Incluye bibliografía e índice
Lopata, Margaret A., Cleveland, Don W., Sollner-Webb, Barbara
Using a plasmid containing the bacterial chloramphenicol acetyl transferase gene, we have assayed for transient expression of DNA introduced into mouse L cells by a variety of transfection...
Millner, Robert J, Brow, MaryAnn D., Cleveland, Don W., Shinick, Thomas M., Sutclffe, J.Gregor
We have determined the 679 nucleotide sequence of a cDNA clone which, by hybridization-translation experiments, corresponds to a 36K chick brain protein. Our studies provide a partial amino add...
Thesis (Ph. D.)--Princeton.
Tubulin requires tau for growth onto microtubule initiating sites (1976)
Witman, George B., Cleveland, Don W., Weingarten, Murray D., Kirschner, Marc W.
Tubulin purified by phosphocellulose chromatography and free of accessory proteins will not form microtubules in the absence or presence of microtubule initiating sites (flagellar microtubules). The...
Pasinelli, Piera, Houseweart, Megan K., Brown, Robert H., Cleveland, Don W.
Familial amyotrophic lateral sclerosis-linked mutations in copper-zinc superoxide dismutase cause motor neuron death through one or more acquired toxic properties. An early event in the mechanism of...
Couillard-Després, Sébastien, Zhu, Qinzhang, Wong, Philip C., Price, Donald L., Cleveland, Don W., Julien, Jean-Pierre
To investigate the role of neurofilaments in motor neuron disease caused by superoxide dismutase (SOD1) mutations, transgenic mice expressing a amyotrophic lateral sclerosis-linked SOD1 mutant...
Williamson, Toni L., Bruijn, Lucie I., Zhu, Qinzhang, Anderson, Karen L., Anderson, Scott D., Julien, Jean-Pierre, ...
Mutations in superoxide dismutase 1 (SOD1), the only proven cause of amyotrophic lateral sclerosis (ALS), provoke disease through an unidentified toxic property. Neurofilament aggregates are...
Duesbery, Nick S., Choi, Taesaeng, Brown, Kevin D., Wood, Kenneth W., Resau, James, Fukasawa, Kenji, ...
CENP-E, a kinesin-like protein that is known to associate with kinetochores during all phases of mitotic chromosome movement, is shown here to be a component of meiotic kinetochores as well. CENP-E...
Corson, Laura B., Strain, Jeffrey J., Culotta, Valeria C., Cleveland, Don W.
Mutations in Cu, Zn superoxide dismutase (SOD1) cause the neurodegenerative disease familial amyotrophic lateral sclerosis from an as-yet-unidentified toxic property(ies). Analysis in Saccharomyces...
Pasinelli, Piera, Borchelt, David R., Houseweart, Megan K., Cleveland, Don W., Brown, Robert H.
The mechanism by which mutations in the superoxide dismutase (SOD1) gene cause motor neuron degeneration in familial amyotrophic lateral sclerosis (ALS) is unknown. Recent reports that neuronal death...
Howland, David S., Liu, Jian, She, Yijin, Goad, Beth, Maragakis, Nicholas J., Kim, Benjamin, ...
Transgenic overexpression of Cu+2/Zn+2 superoxide dismutase 1 (SOD1) harboring an amyotrophic lateral sclerosis (ALS)-linked familial genetic mutation (SOD1G93A) in a Sprague–Dawley rat results in...
Yamanaka, Koji, Vande Velde, Christine, Eymard-Pierre, Eleonore, Bertini, Enrico, Boespflug-Tanguy, Odile, Cleveland, Don W.
Mutations in ALS2, carrying three putative guanine exchange factor (GEF) domains, are causative for a juvenile, autosomal recessive form of amyotrophic lateral sclerosis (ALS), primary lateral...
Foltz, Daniel R., Cleveland, Don W.
A compromised mitotic checkpoint, the primary mechanism for ensuring that each new cell receives one copy of every chromosome, has been implicated as a contributor to carcinogenesis. However, a...
Lobsiger, Christian S., Garcia, Michael L., Ward, Christopher M., Cleveland, Don W.
Eliminating assembled neurofilaments (NFs) from axons or misaccumulating NFs in motor neuron cell bodies strongly slows disease in mouse models of mutant superoxide dismutase 1 (SOD1)-induced...
Virus-Delivered Small RNA Silencing Sustains Strength in Amyotrophic Lateral Sclerosis
Miller, Timothy M., Kaspar, Brian K., Kops, Geert J., Yamanaka, Koji, Christian, Lindsey J., Gage, Fred H., ...
Mutations in superoxide dismutase cause a subset of familial amyotrophic lateral sclerosis and provoke progressive paralysis when expressed in mice. After retrograde transport to the spinal cord...
ZW10 links mitotic checkpoint signaling to the structural kinetochore
Kim, Yumi, Weaver, Beth A.A., Mao, Yinghui, McLeod, Ian, Yates, John R., ...
The mitotic checkpoint ensures that chromosomes are divided equally between daughter cells and is a primary mechanism preventing the chromosome instability often seen in aneuploid human tumors. ZW10...
Antisense oligonucleotide therapy for neurodegenerative disease
Smith, Richard A., Miller, Timothy M., Yamanaka, Koji, Monia, Brett P., Condon, Thomas P., Hung, Gene, ...
Neurotoxicity from accumulation of misfolded/mutant proteins is thought to drive pathogenesis in neurodegenerative diseases. Since decreasing levels of proteins responsible for such accumulations is...
Unstable microtubule capture at kinetochores depleted of the centromere-associated protein CENP-F
Bomont, Pascale, Maddox, Paul, Shah, Jagesh V, Desai, Arshad B, Cleveland, Don W
Centromere protein F (CENP-F) (or mitosin) accumulates to become an abundant nuclear protein in G2, assembles at kinetochores in late G2, remains kinetochore-bound until anaphase, and is degraded at...
Pasinelli, Piera, Houseweart, Megan K., Brown, Robert H., Cleveland, Don W.
Familial amyotrophic lateral sclerosis-linked mutations in copper-zinc superoxide dismutase cause motor neuron death through one or more acquired toxic properties. An early event in the mechanism of...
Couillard-Després, Sébastien, Zhu, Qinzhang, Wong, Philip C., Price, Donald L., Cleveland, Don W., Julien, Jean-Pierre
To investigate the role of neurofilaments in motor neuron disease caused by superoxide dismutase (SOD1) mutations, transgenic mice expressing a amyotrophic lateral sclerosis-linked SOD1 mutant...
Williamson, Toni L., Bruijn, Lucie I., Zhu, Qinzhang, Anderson, Karen L., Anderson, Scott D., Julien, Jean-Pierre, ...
Mutations in superoxide dismutase 1 (SOD1), the only proven cause of amyotrophic lateral sclerosis (ALS), provoke disease through an unidentified toxic property. Neurofilament aggregates are...
Duesbery, Nick S., Choi, Taesaeng, Brown, Kevin D., Wood, Kenneth W., Resau, James, Fukasawa, Kenji, ...
CENP-E, a kinesin-like protein that is known to associate with kinetochores during all phases of mitotic chromosome movement, is shown here to be a component of meiotic kinetochores as well. CENP-E...
Corson, Laura B., Strain, Jeffrey J., Culotta, Valeria C., Cleveland, Don W.
Mutations in Cu, Zn superoxide dismutase (SOD1) cause the neurodegenerative disease familial amyotrophic lateral sclerosis from an as-yet-unidentified toxic property(ies). Analysis in Saccharomyces...
Pasinelli, Piera, Borchelt, David R., Houseweart, Megan K., Cleveland, Don W., Brown, Robert H.
The mechanism by which mutations in the superoxide dismutase (SOD1) gene cause motor neuron degeneration in familial amyotrophic lateral sclerosis (ALS) is unknown. Recent reports that neuronal death...
Howland, David S., Liu, Jian, She, Yijin, Goad, Beth, Maragakis, Nicholas J., Kim, Benjamin, ...
Transgenic overexpression of Cu+2/Zn+2 superoxide dismutase 1 (SOD1) harboring an amyotrophic lateral sclerosis (ALS)-linked familial genetic mutation (SOD1G93A) in a Sprague–Dawley rat results in...
Yamanaka, Koji, Vande Velde, Christine, Eymard-Pierre, Eleonore, Bertini, Enrico, Boespflug-Tanguy, Odile, Cleveland, Don W.
Mutations in ALS2, carrying three putative guanine exchange factor (GEF) domains, are causative for a juvenile, autosomal recessive form of amyotrophic lateral sclerosis (ALS), primary lateral...
Foltz, Daniel R., Cleveland, Don W.
A compromised mitotic checkpoint, the primary mechanism for ensuring that each new cell receives one copy of every chromosome, has been implicated as a contributor to carcinogenesis. However, a...
Lobsiger, Christian S., Garcia, Michael L., Ward, Christopher M., Cleveland, Don W.
Eliminating assembled neurofilaments (NFs) from axons or misaccumulating NFs in motor neuron cell bodies strongly slows disease in mouse models of mutant superoxide dismutase 1 (SOD1)-induced...
Unstable microtubule capture at kinetochores depleted of the centromere-associated protein CENP-F
Bomont, Pascale, Maddox, Paul, Shah, Jagesh V, Desai, Arshad B, Cleveland, Don W
Centromere protein F (CENP-F) (or mitosin) accumulates to become an abundant nuclear protein in G2, assembles at kinetochores in late G2, remains kinetochore-bound until anaphase, and is degraded at...
Antisense oligonucleotide therapy for neurodegenerative disease
Smith, Richard A., Miller, Timothy M., Yamanaka, Koji, Monia, Brett P., Condon, Thomas P., Hung, Gene, ...
Neurotoxicity from accumulation of misfolded/mutant proteins is thought to drive pathogenesis in neurodegenerative diseases. Since decreasing levels of proteins responsible for such accumulations is...
Miller, Timothy M., Kim, Soo H., Yamanaka, Koji, Hester, Mark, Umapathi, Priya, Arnson, Hannah, ...
Amyotrophic lateral sclerosis (ALS) is a fatal, progressive paralysis arising from the premature death of motor neurons. An inherited form is caused by a dominant mutation in the ubiquitously...
Wang, Zifu, Shah, Jagesh V., Berns, Michael W., Cleveland, Don W.
It has been a significant challenge to quantitatively study the dynamic intracellular processes in live cells. These studies are essential for a thorough understanding of the underlying mechanisms...
An epigenetic mark generated by the incorporation of CENP-A into centromeric nucleosomes
Black, Ben E., Brock, Melissa A., Bédard, Sabrina, Woods, Virgil L., Cleveland, Don W.
Mammalian centromeres are defined epigenetically. Although the physical nature of the epigenetic mark is unknown, nucleosomes in which CENP-A replaces histone H3 are at the foundation of centromeric...
Lobsiger, Christian S., Boillée, Séverine, Cleveland, Don W.
Global, age-dependent changes in gene expression from rodent models of inherited ALS caused by dominant mutations in superoxide-dismutase 1 (SOD1) were identified by using gene arrays and RNAs...
Propagation of centromeric chromatin requires exit from mitosis
Jansen, Lars E.T., Black, Ben E., Foltz, Daniel R., Cleveland, Don W.
Centromeres direct chromosomal inheritance by nucleating assembly of the kinetochore, a large multiprotein complex required for microtubule attachment during mitosis. Centromere identity in humans is...
Common molecular signature in SOD1 for both sporadic and familial amyotrophic lateral sclerosis
Gruzman, Arie, Wood, William L., Alpert, Evgenia, Prasad, M. Dharma, Miller, Robert G., Rothstein, Jeffery D., ...
Amyotrophic lateral sclerosis (ALS) is a devastating motor neuron degenerative disease whose etiology and pathogenesis remain poorly understood. Most cases of ALS (≈90%) are sporadic (SALS),...
Revisiting oxidative damage in ALS: microglia, Nox, and mutant SOD1
Boillée, Séverine, Cleveland, Don W.
Mutation in superoxide dismutase–1 (SOD1) causes the inherited degenerative neurological disease familial amyotrophic lateral sclerosis (ALS), a non–cell-autonomous disease: mutant SOD1 synthesis...
ZW10 links mitotic checkpoint signaling to the structural kinetochore
Kim, Yumi, Weaver, Beth A.A., Mao, Yinghui, McLeod, Ian, Yates, John R., ...
The mitotic checkpoint ensures that chromosomes are divided equally between daughter cells and is a primary mechanism preventing the chromosome instability often seen in aneuploid human tumors. ZW10...
A standardized kinesin nomenclature
Lawrence, Carolyn J., Dawe, R. Kelly, Christie, Karen R., Cleveland, Don W., Dawson, Scott C., Endow, Sharyn A., ...
In recent years the kinesin superfamily has become so large that several different naming schemes have emerged, leading to confusion and miscommunication. Here, we set forth a standardized kinesin...
Microtubule capture by CENP-E silences BubR1-dependent mitotic checkpoint signaling
Mao, Yinghui, Desai, Arshad, Cleveland, Don W.
The mitotic checkpoint is the major cell cycle control mechanism for maintaining chromosome content in multicellular organisms. Prevention of premature onset of anaphase requires activation at...
Rao, Mala V., Houseweart, Megan K., Williamson, Toni L., Crawford, Thomas O., Folmer, Janet, Cleveland, Don W.
Neurofilaments are essential for establishment and maintenance of axonal diameter of large myelinated axons, a property that determines the velocity of electrical signal conduction. One prominent...
Yao, Xuebiao, Anderson, Karen L., Cleveland, Don W.
Centromere-associated protein E (CENP-E) is a kinesin-related microtubule motor protein that is essential for chromosome congression during mitosis. Using immunoelectron microscopy, CENP-E is shown...
Abnormal neurofilament transport caused by targeted disruption of neuronal kinesin heavy chain KIF5A
Xia, Chun-Hong, Roberts, Elizabeth A., Her, Lu-Shiun, Liu, Xinran, Williams, David S., Cleveland, Don W., ...
To test the hypothesis that fast anterograde molecular motor proteins power the slow axonal transport of neurofilaments (NFs), we used homologous recombination to generate mice lacking the...
Garcia, Michael L., Lobsiger, Christian S., Shah, Sameer B., Deerinck, Tom J., Crum, John, Young, Darren, ...
Neurofilaments are essential for acquisition of normal axonal calibers. Several lines of evidence have suggested that neurofilament-dependent structuring of axoplasm arises through an...
Weaver, Beth A.A., Bonday, Zahid Q., Putkey, Frances R., Silk, Alain D., Cleveland, Don W.
Centromere-associated protein-E (CENP-E) is an essential mitotic kinesin that is required for efficient, stable microtubule capture at kinetochores. It also directly binds to BubR1, a...
Rao, Mala V., Garcia, Michael L., Miyazaki, Yukio, Gotow, Takahiro, Yuan, Aidong, Mattina, Salvatore, ...
The COOH-terminal tail of mammalian neurofilament heavy subunit (NF-H), the largest neurofilament subunit, contains 44-51 lysine–serine–proline repeats that are nearly stoichiometrically...
Sánchez, Ivelisse, Hassinger, Linda, Sihag, Ram K., Cleveland, Don W., Mohan, Panaiyur, Nixon, Ralph A.
The accumulation of neurofilaments required for postnatal radial growth of myelinated axons is controlled regionally along axons by oligodendroglia. Developmentally regulated processes previously...
Formation of Spindle Poles by Dynein/Dynactin-Dependent Transport of Numa
Merdes, Andreas, Heald, Rebecca, Samejima, Kumiko, Earnshaw, William C., Cleveland, Don W.
NuMA is a large nuclear protein whose relocation to the spindle poles is required for bipolar mitotic spindle assembly. We show here that this process depends on directed NuMA transport toward...
Yucel, Jennifer K., Marszalek, Janet D., McIntosh, J. Richard, Goldstein, Lawrence S.B., Cleveland, Don W., Philp, Alastair Valentine
CENP-meta has been identified as an essential, kinesin-like motor protein in Drosophila. The 257-kD CENP-meta protein is most similar to the vertebrate kinetochore-associated kinesin-like protein...
Jelluma, Nannette, Brenkman, Arjan B., McLeod, Ian, Yates, John R., Cleveland, Don W., Medema, René H., ...
Chang, Yueming, Kong, Qiongman, Shan, Xiu, Tian, Guilian, Ilieva, Hristelina, Cleveland, Don W., ...
Selective association of misfolded ALS-linked mutant SOD1 with the cytoplasmic face of mitochondria
Vande Velde, Christine, Miller, Timothy M., Cashman, Neil R., Cleveland, Don W.
Mutations in copper/zinc superoxide dismutase (SOD1) are causative for dominantly inherited amyotrophic lateral sclerosis (ALS). Despite high variability in biochemical properties among the...
Kim, Yumi, Heuser, John E., Waterman, Clare M., Cleveland, Don W.
The mitotic kinesin centromere protein E (CENP-E) is an essential kinetochore component that directly contributes to the capture and stabilization of spindle microtubules by kinetochores. Although...
Yamanaka, Koji, Boillee, Severine, Roberts, Elizabeth A., Garcia, Michael L., McAlonis-Downes, Melissa, Mikse, Oliver R., ...
Dominant mutations in ubiquitously expressed superoxide dismutase (SOD1) cause familial ALS by provoking premature death of adult motor neurons. To test whether mutant damage to cell types beyond...
Ilieva, Hristelina S., Yamanaka, Koji, Malkmus, Shelle, Kakinohana, Osamu, Yaksh, Tony, Marsala, Martin, ...
Dominant mutations in cytoplasmic dynein (Loa or Cra) have been reported to provoke selective, age-dependent killing of motor neurons, while paradoxically slowing degeneration and death of motor...
Samoshkin, Alexander, Arnaoutov, Alexei, Jansen, Lars E. T., Ouspenski, Ilia, Dye, Louis, Karpova, Tatiana, ...
Condensins I and II in vertebrates are essential ATP-dependent complexes necessary for chromosome condensation in mitosis. Condensins depletion is known to perturb structure and function of...
Requirements for NuMA in maintenance and establishment of mammalian spindle poles
Silk, Alain D., Holland, Andrew J., Cleveland, Don W.
Microtubules of the mitotic spindle in mammalian somatic cells are focused at spindle poles, a process thought to include direct capture by astral microtubules of kinetochores and/or noncentrosomally...
Lobsiger, Christian S., Boillee, Severine, McAlonis-Downes, Melissa, Khan, Amir M., Feltri, M. Laura, Yamanaka, Koji, ...
Neurodegeneration in an inherited form of ALS is non-cell-autonomous, with ALS-causing mutant SOD1 damage developed within multiple cell types. Selective inactivation within motor neurons of an...
Zhong, Zhihui, Ilieva, Hristelina, Hallagan, Lee, Bell, Robert, Singh, Itender, Paquette, Nicole, ...
Activated protein C (APC) is a signaling protease with anticoagulant activity. Here, we have used mice expressing a mutation in superoxide dismutase-1 (SOD1) that is linked to amyotrophic lateral...