Performance and Variability of Local Barley Landraces in Near-Eastern Environments

Homozygous lines from six Syrian and two Jordanian landrace populations were tested under highly productive growing conditions in Tel Hadia (1982/83), under drought stress in Breda (1983/84), and under dryland salinity stress in Hegla (1982/83/84) in Syria. Mean grain yield levels ranged between 260 kg/ha and 4850 kg/ha. Under drought and salinity stress, the majority of the landrace lines out-yielded the best cheeks significantly. In Tel Hadia the check cultivars mostly outyielded the landrace lines, but not always significantly. In all environments the harvest index of the landrace lines was near the optimum for barley. They expressed intermediate plant height and time to flowering, high lodging susceptibility under favorable growing conditions, high protein content, and a wide range of yield component combinations. In the stress environments highly significant genetic variation among the landrace lines was found. The heritabilities for gram yield were high in these trials. The correlations between performance under stress and under favorable growing conditions were poor. Therefore, the largest gains for variety improvement for the Syrian steppe area are expected from direct selection under stress conditions. Unique responses in proline accumulation and germination patterns in saline solution indicated specific adaptation in this material. These landraces, thus, are a useful source of breeding material, which also widens the genetic base of the present breeding program

Clinical and Molecular Aspects of Senataxin Mutations in Amyotrophic Lateral Sclerosis 4

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/154673/1/ana25681_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/154673/2/ana25681.pd

Universal subgap optical conductivity in quasi-one-dimensional Peierls systems

Quasi-one-dimensional Peierls systems with quantum and thermal lattice fluctuations can be modeled by a Dirac-type equation with a Gaussian-correlated off-diagonal disorder. A powerful new method gives the exact disorder-averaged Green function used to compute the optical conductivity. The strong subgap tail of the conductivity has a universal scaling form. The frequency and temperature dependence of the calculated spectrum agrees with experiments on KCP(Br) and trans-polyacetylene.Comment: 11 pages (+ 3 figures), LATEX (REVTEX 3.0

Mechanisms, models and biomarkers in amyotrophic lateral sclerosis

The last 30 years have seen a major advance in the understanding of the clinical and pathological heterogeneity of amyotrophic lateral sclerosis (ALS), and its overlap with frontotemporal dementia. Multiple, seemingly disparate biochemical pathways converge on a common clinical syndrome characterized by progressive loss of upper and lower motor neurons. Pathogenic themes in ALS include excitotoxicity, oxidative stress, mitochondrial dysfunction, neuroinflammation, altered energy metabolism, and most recently RNA mis-processing. The transgenic rodent, overexpressing mutant superoxide dismutase-1, is now only one of several models of ALS pathogenesis. The nematode, fruit fly and zebrafish all offer fresh insight, and the development of induced pluripotent stem cell-derived motor neurons holds promise for the screening of candidate therapeutics. The lack of useful biomarkers in ALS contributes to diagnostic delay, and the inability to stratify patients by prognosis may be an important factor in the failure of therapeutic trials. Biomarkers sensitive to disease activity might lessen reliance on clinical measures and survival as trial endpoints and reduce study length. Emerging proteomic markers of neuronal loss and glial activity in cerebrospinal fluid, a cortical signature derived from advanced structural and functional MRI, and the development of more sensitive measurements of lower motor neuron physiology are leading a new phase of biomarker-driven therapeutic discovery

Exome Sequencing Identifies a Novel TRPV4 Mutation in a CMT2C Family

Molecular and Cellular Biolog

Mechanisms Models and Biomarkers in Amyotrophic Lateral Sclerosis

The last 30 years have seen a major advance in the understanding of the clinical and pathological heterogeneity of amyotrophic lateral sclerosis (ALS), and its overlap with frontotemporal dementia. Multiple, seemingly disparate biochemical pathways converge on a common clinical syndrome characterized by progressive loss of upper and lower motor neurons. Pathogenic themes in ALS include excitotoxicity, oxidative stress, mitochondrial dysfunction, neuroinflammation, altered energy metabolism, and most recently RNA mis-processing. The transgenic rodent, overexpressing mutant superoxide dismutase-1, is now only one of several models of ALS pathogenesis. The nematode, fruit fly and zebrafish all offer fresh insight, and the development of induced pluripotent stem cell-derived motor neurons holds promise for the screening of candidate therapeutics. The lack of useful biomarkers in ALS contributes to diagnostic delay, and the inability to stratify patients by prognosis may be an important factor in the failure of therapeutic trials. Biomarkers sensitive to disease activity might lessen reliance on clinical measures and survival as trial endpoints and reduce study length. Emerging proteomic markers of neuronal loss and glial activity in cerebrospinal fluid, a cortical signature derived from advanced structural and functional MRI, and the development of more sensitive measurements of lower motor neuron physiology are leading a new phase of biomarker-driven therapeutic discovery

A randomized controlled trial of exercise in spinal and bulbar muscular atrophy.

OBJECTIVE: To determine the safety and efficacy of a home-based functional exercise program in spinal and bulbar muscular atrophy (SBMA). METHODS: Subjects were randomly assigned to participate in 12 weeks of either functional exercises (intervention) or a stretching program (control) at the National Institutes of Health in Bethesda, MD. A total of 54 subjects enrolled, and 50 completed the study with 24 in the functional exercise group and 26 in the stretching control group. The primary outcome measure was the Adult Myopathy Assessment Tool (AMAT) total score, and secondary measures included total activity by accelerometry, muscle strength, balance, timed up and go, sit-to-stand test, health-related quality of life, creatine kinase, and insulin-like growth factor-1. RESULTS: Functional exercise was well tolerated but did not lead to significant group differences in the primary outcome measure or any of the secondary measures. The functional exercise did not produce significantly more adverse events than stretching, and was not perceived to be difficult. To determine whether a subset of the subjects may have benefited, we divided them into high and low functioning based on baseline AMAT scores and performed a post hoc subgroup analysis. Low-functioning individuals receiving the intervention increased AMAT functional subscale scores compared to the control group. INTERPRETATION: Although these trial results indicate that functional exercise had no significant effect on total AMAT scores or on mobility, strength, balance, and quality of life, post hoc findings indicate that low-functioning men with SBMA may respond better to functional exercises, and this warrants further investigation with appropriate exercise intensity

A motor neuron disease–associated mutation in p150Glued perturbs dynactin function and induces protein aggregation

The microtubule motor cytoplasmic dynein and its activator dynactin drive vesicular transport and mitotic spindle organization. Dynactin is ubiquitously expressed in eukaryotes, but a G59S mutation in the p150Glued subunit of dynactin results in the specific degeneration of motor neurons. This mutation in the conserved cytoskeleton-associated protein, glycine-rich (CAP-Gly) domain lowers the affinity of p150Glued for microtubules and EB1. Cell lines from patients are morphologically normal but show delayed recovery after nocodazole treatment, consistent with a subtle disruption of dynein/dynactin function. The G59S mutation disrupts the folding of the CAP-Gly domain, resulting in aggregation of the p150Glued protein both in vitro and in vivo, which is accompanied by an increase in cell death in a motor neuron cell line. Overexpression of the chaperone Hsp70 inhibits aggregate formation and prevents cell death. These data support a model in which a point mutation in p150Glued causes both loss of dynein/dynactin function and gain of toxic function, which together lead to motor neuron cell death

Effect of disorder on quantum phase transitions in anisotropic XY spin chains in a transverse field

We present some exact results for the effect of disorder on the critical properties of an anisotropic XY spin chain in a transverse field. The continuum limit of the corresponding fermion model is taken and in various cases results in a Dirac equation with a random mass. Exact analytic techniques can then be used to evaluate the density of states and the localization length. In the presence of disorder the ferromagnetic-paramagnetic or Ising transition of the model is in the same universality class as the random transverse field Ising model solved by Fisher using a real space renormalization group decimation technique (RSRGDT). If there is only randomness in the anisotropy of the magnetic exchange then the anisotropy transition (from a ferromagnet in the $x$ direction to a ferromagnet in the $y$ direction) is also in this universality class. However, if there is randomness in the isotropic part of the exchange or in the transverse field then in a non-zero transverse field the anisotropy transition is destroyed by the disorder. We show that in the Griffiths' phase near the Ising transition that the ground state energy has an essential singularity. The results obtained for the dynamical critical exponent, the typical correlation length, and the temperature dependence of the specific heat near the Ising transition agree with the results of the RSRGDT and numerical work.Comment: 22 pages, RevTeX + epsf, 4 figure