Vascular Defects and Spinal Cord Hypoxia in Spinal Muscular Atrophy

Motor neuron cell-nonautonomous rescue of spinal muscular atrophy phenotypes in mild and severe transgenic mouse models

(2015) Yimin Hua et al.   GENES & DEVELOPMENT

Repeated low doses of morpholino antisense oligomer: an intermediate mouse model of spinal muscular atrophy to explore the window of therapeutic response

(2015) Haiyan Zhou et al.   HUMAN MOLECULAR GENETICS

Abbreviated Exposure to Hypoxia Is Sufficient to Induce CNS Dysmyelination, Modulate Spinal Motor Neuron Composition, and Impair Motor Development in Neonatal Mice

(2015) Jens O. Watzlawik et al.   PLoS One

Dysregulation of ubiquitin homeostasis and β-catenin signaling promote spinal muscular atrophy

(2014) Thomas M. Wishart et al.   JOURNAL OF CLINICAL INVESTIGATION

Sodium vanadate combined with l-ascorbic acid delays disease progression, enhances motor performance, and ameliorates muscle atrophy and weakness in mice with spinal muscular atrophy

(2013) Huei-Chun Liu et al.   BMC Medicine

Chondrolectin affects cell survival and neuronal outgrowth in in vitro and in vivo models of spinal muscular atrophy

(2013) James N. Sleigh et al.   HUMAN MOLECULAR GENETICS

ALS and Oxidative Stress: The Neurovascular Scenario

(2013) Akshay Anand et al.   Oxidative Medicine and Cellular Longevity

Improvement of Neuromuscular Synaptic Phenotypes without Enhanced Survival and Motor Function in Severe Spinal Muscular Atrophy Mice Selectively Rescued in Motor Neurons

(2013) Ximena Paez-Colasante et al.   PLoS One

Intermittent Hypoxia Can Aggravate Motor Neuronal Loss and Cognitive Dysfunction in ALS Mice

(2013) Sung-Min Kim et al.   PLoS One

Impaired blood–brain/spinal cord barrier in ALS patients

(2012) Svitlana Garbuzova-Davis et al.   BRAIN RESEARCH

Characterization of behavioral and neuromuscular junction phenotypes in a novel allelic series of SMA mouse models

(2012) Melissa Osborne et al.   HUMAN MOLECULAR GENETICS

Hypoxia is a modifier of SMN2 splicing and disease severity in a severe SMA mouse model

(2012) Thomas W. Bebee et al.   HUMAN MOLECULAR GENETICS

Hypoxia Markers are Expressed in Interneurons Exposed to Recurrent Seizures

(2012) Fabio Gualtieri et al.   NEUROMOLECULAR MEDICINE

Limited Phenotypic Effects of Selectively Augmenting the SMN Protein in the Neurons of a Mouse Model of Severe Spinal Muscular Atrophy

(2012) Andrew J-H. Lee et al.   PLoS One

Spinal muscular atrophy: going beyond the motor neuron

(2012) Gillian Hamilton et al.   TRENDS IN MOLECULAR MEDICINE

The contribution of mouse models to understanding the pathogenesis of spinal muscular atrophy

(2011) J. N. Sleigh et al.   Disease Models & Mechanisms

The response of neuromuscular junctions to injury is developmentally regulated

(2011) Lyndsay M. Murray et al.   FASEB JOURNAL

Why is the partial oxygen pressure of human tissues a crucial parameter? Small molecules and hypoxia

(2011) Aude Carreau et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Density, calibre and ramification of muscle capillaries are altered in a mouse model of severe spinal muscular atrophy

(2011) E. Somers et al.   NEUROMUSCULAR DISORDERS

Scaling of Brain Metabolism with a Fixed Energy Budget per Neuron: Implications for Neuronal Activity, Plasticity and Evolution

(2011) Suzana Herculano-Houzel   PLoS One

SAHA ameliorates the SMA phenotype in two mouse models for spinal muscular atrophy

(2010) Markus Riessland et al.   HUMAN MOLECULAR GENETICS

Arrhythmia and cardiac defects are a feature of spinal muscular atrophy model mice

(2010) Christopher R. Heier et al.   HUMAN MOLECULAR GENETICS

Cardiac defects contribute to the pathology of spinal muscular atrophy models

(2010) Monir Shababi et al.   HUMAN MOLECULAR GENETICS

Early heart failure in the SMNΔ7 model of spinal muscular atrophy and correction by postnatal scAAV9-SMN delivery

(2010) Adam K. Bevan et al.   HUMAN MOLECULAR GENETICS

Digital necroses and vascular thrombosis in severe spinal muscular atrophy

(2010) Sabine Rudnik-Schöneborn et al.   MUSCLE & NERVE

Significance of nitroimidazole compounds and hypoxia-inducible factor-1 for imaging tumor hypoxia

(2009) Shinae Kizaka-Kondoh et al.   CANCER SCIENCE

Spinal muscular atrophy: why do low levels of survival motor neuron protein make motor neurons sick?

(2009) Arthur H. M. Burghes et al.   NATURE REVIEWS NEUROSCIENCE

Structure and function of the blood–brain barrier

(2009) N. Joan Abbott et al.   NEUROBIOLOGY OF DISEASE

Alternative Splicing Events Are a Late Feature of Pathology in a Mouse Model of Spinal Muscular Atrophy

(2009) Dirk Bäumer et al.   PLoS Genetics

Rapid loss of motor nerve terminals following hypoxia–reperfusion injury occurs via mechanisms distinct from classic Wallerian degeneration

(2008) Becki Baxter et al.   JOURNAL OF ANATOMY

Inactivation of the SMN Complex by Oxidative Stress

(2008) Lili Wan et al.   MOLECULAR CELL

ALS-causing SOD1 mutants generate vascular changes prior to motor neuron degeneration

(2008) Zhihui Zhong et al.   NATURE NEUROSCIENCE