Published in J Comp Neurol on February 01, 2006
Sumo1 conjugates mitochondrial substrates and participates in mitochondrial fission. Curr Biol (2004) 3.21
Cargo-selected transport from the mitochondria to peroxisomes is mediated by vesicular carriers. Curr Biol (2008) 2.67
Activated mitofusin 2 signals mitochondrial fusion, interferes with Bax activation, and reduces susceptibility to radical induced depolarization. J Biol Chem (2005) 2.57
A vesicular transport pathway shuttles cargo from mitochondria to lysosomes. Curr Biol (2012) 2.45
The SUMO protease SENP5 is required to maintain mitochondrial morphology and function. J Cell Sci (2007) 2.12
Chx10 repression of Mitf is required for the maintenance of mammalian neuroretinal identity. Development (2004) 1.79
Dissociating the dual roles of apoptosis-inducing factor in maintaining mitochondrial structure and apoptosis. EMBO J (2006) 1.59
SRp30c-dependent stimulation of survival motor neuron (SMN) exon 7 inclusion is facilitated by a direct interaction with hTra2 beta 1. Hum Mol Genet (2002) 1.53
A 1.3 kb promoter fragment confers spatial and temporal expression of utrophin A mRNA in mouse skeletal muscle fibers. Neuromuscul Disord (2005) 1.45
Transgenic expression of the activating natural killer receptor Ly49H confers resistance to cytomegalovirus in genetically susceptible mice. J Exp Med (2003) 1.37
Reconstitution of mitochondria derived vesicle formation demonstrates selective enrichment of oxidized cargo. PLoS One (2012) 1.27
Rho-kinase inactivation prolongs survival of an intermediate SMA mouse model. Hum Mol Genet (2010) 1.27
SMN, profilin IIa and plastin 3: a link between the deregulation of actin dynamics and SMA pathogenesis. Mol Cell Neurosci (2009) 1.26
Hypomorphic Smn knockdown C2C12 myoblasts reveal intrinsic defects in myoblast fusion and myotube morphology. Exp Cell Res (2005) 1.23
A critical smn threshold in mice dictates onset of an intermediate spinal muscular atrophy phenotype associated with a distinct neuromuscular junction pathology. Neuromuscul Disord (2011) 1.20
Glucose metabolism and pancreatic defects in spinal muscular atrophy. Ann Neurol (2012) 1.17
Neurodevelopmental defects resulting from ATRX overexpression in transgenic mice. Hum Mol Genet (2002) 1.13
Smn depletion alters profilin II expression and leads to upregulation of the RhoA/ROCK pathway and defects in neuronal integrity. J Mol Neurosci (2007) 1.12
Mouse survival motor neuron alleles that mimic SMN2 splicing and are inducible rescue embryonic lethality early in development but not late. PLoS One (2010) 1.11
Derivation of enriched oligodendrocyte cultures and oligodendrocyte/neuron myelinating co-cultures from post-natal murine tissues. J Vis Exp (2011) 1.10
The myogenic kinome: protein kinases critical to mammalian skeletal myogenesis. Skelet Muscle (2011) 1.10
Fasudil improves survival and promotes skeletal muscle development in a mouse model of spinal muscular atrophy. BMC Med (2012) 1.05
Dominant-negative beta1 integrin mice have region-specific myelin defects accompanied by alterations in MAPK activity. Glia (2006) 1.04
A novel cell-free mitochondrial fusion assay amenable for high-throughput screenings of fusion modulators. BMC Biol (2010) 1.04
Characterization of liver histopathology in a transgenic mouse model expressing genotype 1a hepatitis C virus core and envelope proteins 1 and 2. J Gen Virol (2005) 1.04
Microtubule stability, Golgi organization, and transport flux require dystonin-a2-MAP1B interaction. J Cell Biol (2012) 1.03
Wnt11 promotes cardiomyocyte development by caspase-mediated suppression of canonical Wnt signals. Mol Cell Biol (2010) 1.02
Disruption of MEF2 activity in cardiomyoblasts inhibits cardiomyogenesis. J Cell Sci (2006) 0.99
Dystonin/Bpag1--a link to what? Cell Motil Cytoskeleton (2007) 0.99
Biochemical, proteomic, structural, and thermodynamic characterizations of integrin-linked kinase (ILK): cross-validation of the pseudokinase. J Biol Chem (2011) 0.99
A SMNDelta7 read-through product confers functionality to the SMNDelta7 protein. Neurosci Lett (2008) 0.97
Dystonin/Bpag1 is a necessary endoplasmic reticulum/nuclear envelope protein in sensory neurons. Exp Cell Res (2008) 0.97
Cdx2 regulation of posterior development through non-Hox targets. Development (2009) 0.96
Conservation, variability and the modeling of active protein kinases. PLoS One (2007) 0.95
A novel function for the survival motoneuron protein as a translational regulator. Hum Mol Genet (2012) 0.95
Spectrin repeat proteins in the nucleus. Bioessays (2005) 0.94
Genetic alterations at the Bpag1 locus in dt mice and their impact on transcript expression. Mamm Genome (2005) 0.94
Early onset muscle weakness and disruption of muscle proteins in mouse models of spinal muscular atrophy. Skelet Muscle (2013) 0.94
A Bpag1 isoform involved in cytoskeletal organization surrounding the nucleus. Exp Cell Res (2005) 0.94
The Rb/E2F pathway modulates neurogenesis through direct regulation of the Dlx1/Dlx2 bigene cluster. J Neurosci (2012) 0.93
Characterization of transgene expression and Cre recombinase activity in a panel of Thy-1 promoter-Cre transgenic mice. Dev Dyn (2002) 0.93
Bin1 SRC homology 3 domain acts as a scaffold for myofiber sarcomere assembly. J Biol Chem (2009) 0.90
Calpain-mediated processing of p53-associated parkin-like cytoplasmic protein (PARC) affects chemosensitivity of human ovarian cancer cells by promoting p53 subcellular trafficking. J Biol Chem (2011) 0.89
Bpag1 localization to actin filaments and to the nucleus is regulated by its N-terminus. J Cell Sci (2003) 0.88
Identification of novel interacting protein partners of SMN using tandem affinity purification. J Proteome Res (2010) 0.88
Retargeting of adenovirus vectors through genetic fusion of a single-chain or single-domain antibody to capsid protein IX. J Virol (2010) 0.88
A novel whole-cell lysate kinase assay identifies substrates of the p38 MAPK in differentiating myoblasts. Skelet Muscle (2012) 0.88
Neuronal dystonin isoform 2 is a mediator of endoplasmic reticulum structure and function. Mol Biol Cell (2011) 0.88
Smn deficiency causes neuritogenesis and neurogenesis defects in the retinal neurons of a mouse model of spinal muscular atrophy. Dev Neurobiol (2011) 0.88
Neurodevelopmental consequences of Smn depletion in a mouse model of spinal muscular atrophy. J Neurosci Res (2010) 0.88
Integrin signaling in oligodendrocytes and its importance in CNS myelination. J Signal Transduct (2010) 0.87
Neurodevelopmental abnormalities in neurosphere-derived neural stem cells from SMN-depleted mice. J Neurosci Res (2008) 0.87
Six1 regulates MyoD expression in adult muscle progenitor cells. PLoS One (2013) 0.87
Untethering the nuclear envelope and cytoskeleton: biologically distinct dystonias arising from a common cellular dysfunction. Int J Cell Biol (2012) 0.87
Physiological maturation of photoreceptors depends on the voltage-gated sodium channel NaV1.6 (Scn8a). J Neurosci (2005) 0.86
The proteolipid protein promoter drives expression outside of the oligodendrocyte lineage during embryonic and early postnatal development. PLoS One (2011) 0.86
Matrix protein of Vesicular stomatitis virus harbours a cryptic mitochondrial-targeting motif. J Gen Virol (2006) 0.86
The interaction and cellular localization of HSP27 and ERbeta are modulated by 17beta-estradiol and HSP27 phosphorylation. Mol Cell Endocrinol (2007) 0.85
MAP1B and clathrin are novel interacting partners of the giant cyto-linker dystonin. J Proteome Res (2011) 0.85
Specific contribution of lamin A and lamin C in the development of laminopathies. Exp Cell Res (2008) 0.85
Acquisition of arylsulfatase A onto the mouse sperm surface during epididymal transit. Biol Reprod (2003) 0.84
Integrin-linked kinase regulates process extension in oligodendrocytes via control of actin cytoskeletal dynamics. J Neurosci (2013) 0.84
SR-BI undergoes cholesterol-stimulated transcytosis to the bile canaliculus in polarized WIF-B cells. J Biol Chem (2006) 0.84
Motor unit abnormalities in Dystonia musculorum mice. PLoS One (2011) 0.84
Dissection of the transversus abdominis muscle for whole-mount neuromuscular junction analysis. J Vis Exp (2014) 0.84
The role of conserved water molecules in the catalytic domain of protein kinases. Proteins (2009) 0.83
The dynamics of cardiolipin synthesis post-mitochondrial fusion. Biochim Biophys Acta (2010) 0.82
Hearts of dystonia musculorum mice display normal morphological and histological features but show signs of cardiac stress. PLoS One (2010) 0.82
Cerebral ischemia causes dysregulation of synaptic adhesion in mouse synaptosomes. J Cereb Blood Flow Metab (2007) 0.82
Development of a gene therapy strategy for the restoration of survival motor neuron protein expression: implications for spinal muscular atrophy therapy. Hum Gene Ther (2003) 0.82
Cellular and molecular biology of neuronal dystonin. Int Rev Cell Mol Biol (2013) 0.81
Impaired fast axonal transport in neurons of the sciatic nerves from dystonia musculorum mice. J Neurochem (2003) 0.81
A novel role for the cytoskeletal linker protein dystonin in the maintenance of microtubule stability and the regulation of ER-Golgi transport. Bioarchitecture (2012) 0.81
Production of mouse chimeras by injection of embryonic stem cells into the perivitelline space of one-cell stage embryos. Transgenic Res (2010) 0.80
Mitochondrial dynamics and physiology. Biochim Biophys Acta (2012) 0.80
Disruption in the autophagic process underlies the sensory neuropathy in dystonia musculorum mice. Autophagy (2015) 0.79
Mice with podocyte-specific overexpression of wild type alpha-actinin-4 are healthy controls for K256E-alpha-actinin-4 mutant transgenic mice. Transgenic Res (2009) 0.79
The mouse dystrophin muscle promoter/enhancer drives expression of mini-dystrophin in transgenic mdx mice and rescues the dystrophy in these mice. Mol Ther (2006) 0.79
Variable effects of gender and Western diet on lipid and glucose homeostasis in aged PCSK9-deficient C57BL/6 mice CSK9PC57BL/6. J Diabetes (2014) 0.79
Nanosilver cytotoxicity in rainbow trout (Oncorhynchus mykiss) erythrocytes and hepatocytes. Comp Biochem Physiol C Toxicol Pharmacol (2013) 0.79
Biochemical and cellular analysis of human variants of the DYT1 dystonia protein, TorsinA/TOR1A. Hum Mutat (2014) 0.79
Supraphysiological expression of survival motor neuron protein from an adenovirus vector does not adversely affect cell function. Biochem Cell Biol (2013) 0.78
Use of Cre/loxP recombination to swap cell binding motifs on the adenoviral capsid protein IX. Virology (2011) 0.78
Transgenic expression of neuronal dystonin isoform 2 partially rescues the disease phenotype of the dystonia musculorum mouse model of hereditary sensory autonomic neuropathy VI. Hum Mol Genet (2013) 0.78
Differentiation potential of primary myogenic cells derived from skeletal muscle of dystonia musculorum mice. Differentiation (2002) 0.78
The mouse dystrophin muscle enhancer-1 imparts skeletal muscle, but not cardiac muscle, expression onto the dystrophin Purkinje promoter in transgenic mice. Hum Mol Genet (2004) 0.78
Effects of nitric oxide donors on cybrids harbouring the mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) A3243G mitochondrial DNA mutation. Biochem J (2005) 0.78
Alterations in myelination in the central nervous system of dystonia musculorum mice. J Neurosci Res (2002) 0.77
Active kinase proteome screening reveals novel signal complexity in cardiomyopathy. Mol Cell Proteomics (2005) 0.77
Reduction of neuronal intranuclear rodlets immunoreactive for tubulin and glucocorticoid receptor in Alzheimer's disease. Brain Pathol (2002) 0.77
Re: "A possible cellular mechanism of neuronal loss in the dorsal root ganglia of dystonia musculorum (dt) mice". J Neuropathol Exp Neurol (2007) 0.76
Distinct roles for Ste20-like kinase SLK in muscle function and regeneration. Skelet Muscle (2013) 0.76
Heidi McBride: mitochondria are well connected. J Cell Biol (2014) 0.75
Corrigendum: Establishment of a cone photoreceptor transplantation platform based on a novel cone-GFP reporter mouse line. Sci Rep (2016) 0.75
In memoriam: Stewart Charles Whitman (1964-2010). J Lipid Res (2010) 0.75
Mouse dystrophin enhancer preferentially targets lacZ expression in skeletal and cardiac muscle. Dev Dyn (2002) 0.75