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PubMedDate not supplied

Prospective study of spinal muscular atrophy before age 6 years. DCN/SMA Group.

Spinal muscular atrophy (SMA) is a common neuromuscular disorder of childhood, associated with a high mortality rate during the first 2 years of life. Most practitioners expect patients with SMA to follow a progressive course with loss of muscle strength and function over 2-10 years. Counselling sessions with parents frequently emphasize the high mortality rate and risk for respiratory failure. The progressive nature of SMA has been attributed to the loss of motor neurons. Fifty-eight children, ages 6 years and younger, were examined between January, 1987, and April, 1992, as part of a large, multicenter collaborative study of SMA. Muscle function was evaluated at regular intervals using a standardized protocol that was demonstrated to be reliable. We determined a prevalence of 56% for tongue fasciculations, a prevalence of 22% for facial weakness, and persistent deep tendon reflexes in one patient. Improved motor function and acquired milestones during the study were documented. This work should contribute toward a better understanding of the natural history of SMA.

Child↗
PubMedDate not supplied

Patterns of Schwann cell myelination of axons within the spinal cord.

Patterns of Schwann cell myelination of long-projecting axons in the spinal cord were studied. The goal was to determine if such axons arising from neurons whose somata and processes are normally confined to the central nervous system can interact effectively with Schwann cells, the myelinating cells of the peripheral nervous system. In one paradigm Schwann cells develop in the dorsal funiculi of the lumbar spinal cord subsequent to radiation-induced alterations in development of the glial populations. Light and electron microscopic evaluations were made in the region of the corticospinal tracts (CSTs), which in the rat occupy the base of the dorsal funiculi. At 90 days following irradiation, larger axons of these tracts (> 1.5 microns in diameter) were myelinated by Schwann cells, and smaller axons were ensheathed by them. In the second paradigm cultured Schwann cells were injected into the medial portions of the ventral funiculi at 13 days post-irradiation when the glial population was markedly reduced. Earlier investigations from this laboratory demonstrated that Schwann cells do not develop in the irradiated ventral funiculi, as they do dorsally. When placed in proximity to long-projecting axons in the medial portion of the ventral funiculi, the Schwann cells either formed compact myelin sheaths or ensheathed axons, depending upon their diameter. Fasciculation and presence of collagen were characteristic of this paradigm but were absent from the Schwann cell-occupied regions of the CSTs. This probably relates to the presence of fibroblasts in the injected cultures.(ABSTRACT TRUNCATED AT 250 WORDS)

Animals↗
PubMed1988

Spatial regulation of axonal glycoprotein expression on subsets of embryonic spinal neurons.

The identification of surface proteins restricted to subsets of embryonic axons and growth cones may provide information on the mechanisms underlying axon fasciculation and pathway selection in the vertebrate nervous system. We describe here the characterization of a 135 kd cell surface glycoprotein, TAG-1, that is expressed transiently on subsets of embryonic spinal cord axons and growth cones. TAG-1 is immunochemically distinct from the cell adhesion molecules N-CAM and L1 (NILE) and is expressed on commissural and motor neurons over the period of initial axon extension. Moreover, TAG-1 and L1 appear to be segregated on different segments of the same embryonic spinal axons. These observations provide evidence that axonal guidance and pathway selection in vertebrates may be regulated in part by the transient and selective expression of distinct surface glycoproteins on subsets of developing neurons.

Animals↗
PubMed1989

Neural cell recognition molecule F11: homology with fibronectin type III and immunoglobulin type C domains.

We report here the complete cDNA sequence of F11 130 kd polypeptide, a chick neural cell surface-associated glycoprotein implicated in neurite fasciculation and elongation. The predicted protein sequence of 1010 amino acids includes an amino-terminal signal peptide and a carboxy-terminal hydrophobic stretch, which is compatible with the consensus motif for covalent attachment of glycosyl-phosphatidylinositol. Accordingly, F11 lacks an intracellular domain, which is consistent with evidence obtained from protease protection experiments on isolated microsomes. In addition, the molecule comprises six domains related to the immunoglobulin domain type C and four resembling fibronectin repeat type III. Both types of repeats resemble those present in neural cell adhesion molecules L1 and N-CAM. The possible identity of F11 with the chick neural glycoprotein contactin is discussed.

Amino Acid Sequence↗
PubMed1989

The growth cones of identified motoneurons in embryonic zebrafish select appropriate pathways in the absence of specific cellular interactions.

Developing motoneurons in zebrafish embryos follow a stereotyped sequence of axonal outgrowth and accurately project their axons to cell-specific target muscles. During axonal pathfinding, an identified motoneuron pioneers the peripheral motor pathway. Growth cones of later motoneurons interact with the pioneer via contact, coupling, and axonal fasciculation. In spite of these interactions, ablation of the pioneer motoneuron does not affect the ability of other identified motoneurons to select the pathways that lead to appropriate target muscles. We conclude that interactions between these cells during pathfinding are not required for accurate pathway selection.

Animals↗
PubMed1989

Defasciculation as a neuronal pathfinding strategy: involvement of a specific glycoprotein.

Leech sensory afferents change their growth behavior as they enter the CNS. Arriving from the periphery in fasciculated tracts, they abruptly defasciculate and expand into diffuse trees before reassembling into four distinct central tracts. In the organ-cultured germinal plate, growing sensory afferents were incubated with monovalent Fab fragments of the Lan3-2 antibody, which recognizes a 130 kd sensory neuron protein by its mannose epitope. Very low concentrations of Lan3-2 (6 and 12 nM) specifically inhibited the central defasciculation of sensory afferents, which then continued growing as a single tract. In contrast, monoclonal antibody Lan3-6, which binds to an internal sensory antigen, failed to yield the same effect. These observations suggest that this sensory neuron 130 kd surface glycoprotein participates in a developmentally significant heterophilic interaction specific for the CNS.

Afferent Pathways↗
PubMed1990

Polysialic acid as a regulator of intramuscular nerve branching during embryonic development.

The role of polysialic acid (PSA) during initial innervation of chick muscle was examined. Previously, the adhesion molecules L1 and N-CAM were shown to be important in balancing axon-axon and axon-muscle adhesion during this process. Here we demonstrate developmental changes in the pattern of innervation that are not correlated with levels of L1 or N-CAM expression, but rather with the amount of PSA at the axon surface. Removal of PSA by a specific endoneuraminidase (Endo-N) increased axon fasciculation and reduced nerve branching. In contrast, the nerve trunk defasciculation and increased branching produced by neuromuscular activity blockade were associated with an increase in axonal PSA levels. Furthermore, Endo-N prevented these inactivity-induced effects on branching. Together these results illustrate the potential of PSA as a regulator of cell-cell interactions and provide a direct example of a molecular link between the morphogenic effects of adhesion-mediated and synaptic activity-dependent processes.

Animals↗
PubMed1991

Molecular mechanisms separating two axonal pathways during embryonic development of the avian optic tectum.

During embryonic development of the avian optic tectum, retinal and tectobulbar axons form an orthogonal array of nerve processes. Growing axons of both tracts are transiently very closely apposed to each other. Despite this spatial proximity, axons from the two pathways do not intermix, but instead restrict their growth to defined areas, thus forming two separate plexiform layers, the stratum opticum and the stratum album centrale. In this study we present experimental evidence indicating that the following three mechanisms might play a role in segregating both axonal populations: Retinal and tectobulbar axons differ in their ability to use the extracellular matrix protein laminin as a substrate for axonal elongation; the environment in the optic tectum is generally permissive for retinal axons, but is specifically nonpermissive for tectobulbar axons, resulting in a strong fasciculation of the latter; and growth cones of temporal retinal axons are reversibly inhibited in their motility by direct contact with the tectobulbar axon's membrane.

Animals↗
PubMed1991

Molecular cloning and expression of a novel adhesion molecule, SC1.

SC1, an integral membrane glycoprotein of 100 kd, is uniquely and transiently expressed on spinal cord motoneurons early in development and appears in peripheral neurons and several other tissues during development. SC1 has been purified by immunoaffinity techniques, and SC1 cDNA clones have been obtained by screening an E4 chick embryo phage expression library with a rabbit polyclonal antibody produced against purified SC1. The deduced protein sequence of 588 amino acids consists of a signal peptide, five immunoglobulin-like domains, a transmembrane region, and a short cytoplasmic tail. The sequence is most similar to MUC18, reported as a tumor progression marker in human melanoma. Transfection of SC1 cDNA into mammalian cells leads to cell surface expression of SC1 antigen and a subsequent increase in cell-cell adhesion. SC1 molecules bind to each other via a homophilic adhesion mechanism, independently of calcium or magnesium ions. SC1 may have a role in lateral motor column formation or neurite growth or fasciculation.

Activated-Leukocyte Cell Adhesion Molecule↗
PubMed1992

Role of the floor plate in axonal patterning in the zebrafish CNS.

To determine the role of the floor plate (FP) in CNS development, I have used labeling techniques, including immunolabeling, to analyze cyclops mutant embryos, which lack the FP. Except for the anterior brain, the mutant phenotype is almost exclusively confined to the vicinity of the ventral CNS midline. In the midbrain, the number of ventral neurons is reduced and cell patterning is disturbed. In contrast, the neuronal arrangement in the spinal cord is almost normal, including in particular both primary and secondary motoneurons. Longitudinal axonal bundles are disorganized in both the brain and spinal cord. Laser ablating the FP in wild-type embryos locally phenocopies cyclops axonal disturbances, and transplanting wild-type FP precursor cells into mutants locally rescues the disturbances. These results demonstrate a significant role for the FP in pathfinding and fasciculation by axons in situ, especially during their longitudinal courses.

Animals↗
PubMed1992

Formation of the Drosophila larval photoreceptor organ and its neuronal differentiation require continuous Krüppel gene activity.

The Drosophila segmentation gene Krüppel (Kr) is redeployed to play a critical role for the establishment of the larval visual system. Using reporter gene expression conducted by a specific Kr cis-acting element, we were able to trace back the origin of the larval photoreceptor organ, the Bolwig organ, to a single progenitor neuron and to examine Kr function in Bolwig organ development when Kr+ activity is absent from embryos due to specific mutations or reduced by neuron-specific and temporally restricted Kr antisense RNA expression. Our results show that Kr is required for neurons to differentiate into Bolwig organs, for fasciculation of the Bolwig nerve, and for this nerve to follow a specific pathway toward the synaptic targets in the larval brain. The transcription factor encoded by Kr is likely to regulate surface molecules necessary for neuronal cell adhesion and recognition in the developing larval visual system.

Animals↗
PubMed1993

Neuromusculin, a Drosophila gene expressed in peripheral neuronal precursors and muscles, encodes a cell adhesion molecule.

To unravel the molecular mechanisms of peripheral nervous system differentiation in Drosophila, we have screened for and identified genes that are expressed in sensory mother cells. Here, we describe a novel gene, neuromusculin (nrm), that is expressed in sensory mother cells and developing muscles. nrm encodes a member of the immunoglobulin superfamily. Immunoblots of Schneider 2 cells transfected with an nrm cDNA indicate that Nrm is present in a membrane-associated form and a secreted form. Cell aggregation assays suggest that Nrm is a homophilic cell adhesion molecule that is secreted or released after proteolysis, a mechanism that to our knowledge has not been described for immunoglobulin-like molecules. Genetic analyses indicate that nrm is an essential gene required for larval viability. We propose that Nrm may play a role as a cell adhesion molecule in clustering cells of the peripheral nervous system, neuronal fasciculation, and/or pathfinding.

Amino Acid Sequence↗
PubMed1993

Fasciclin I and II have distinct roles in the development of grasshopper pioneer neurons.

We have used a new technique, micro-CALI (chromophore-assisted laser inactivation), to investigate the function of the neural cell adhesion molecules fasciclin I and II in the development of the grasshopper Ti1 neurons. Micro-CALI of fasciclin I results in defasciculation of the Ti1 axons similar to that achieved using large scale CALI (Jay and Keshishian, 1990). The initial point of axon separation corresponds to the site of laser irradiation, and defasciculation always continues distal to this point. Micro-CALI of fasciclin II prevents the initiation of Ti1 axon outgrowth but has no effect on fasciculation. This effect is restricted to a 3 hr interval between cytokinesis and growth cone emergence.

Animals↗
PubMed1995

Crossed and uncrossed retinal axons respond differently to cells of the optic chiasm midline in vitro.

In mouse, retinal axon divergence takes place within a cellular specialization localized at the midline of the optic chiasm. To test whether the cells in this locus present cues for differential retinal axon growth, retinal explants were cocultured with cells dissociated from the chiasmatic midline, both taken from day 14-15 embryos, during the principal period of retinal axon divergence. Compared with crossed axons from other retinal regions, axons from ventrotemporal retina, the sole source of uncrossed axons, were shorter, more fasciculated, and fewer in number when growing on chiasm cells. Furthermore, uncrossed axons avoided clusters of chiasm neurons and glia having the composition and arrangement of the midline specialization, but crossed axons readily grew over them. In contrast to the clusters of chiasm cells, however, individual neurons and glia did not elicit differential retinal axon growth. These data demonstrate that cues for divergence derive from cells resident to the chiasm and suggest that cellular interactions among resident midline cells are required to produce these cues.

Animals↗
PubMed1995

GDNF prevents degeneration and promotes the phenotype of brain noradrenergic neurons in vivo.

The locus coeruleus (LC), the main noradrenergic center in the brain, participates in many neural functions, as diverse as memory and motor output, and is severely affected in several neurodegenerative disorders of the CNS. GDNF, a neurotrophic factor initially identified as dopaminotrophic, was found to be expressed in several targets of central noradrenergic neurons in the adult rat brain. Grafting of genetically engineered fibroblasts expressing high levels of GDNF prevented > 80% of the 6-hydroxydopamine-induced degeneration of noradrenergic neurons in the LC in vivo. Moreover, GDNF induced a fasciculated sprouting and increased by 2.5-fold both tyrosine hydroxylase levels and the soma size of lesioned LC neurons. These findings reveal a novel and potent neurotrophic activity of GDNF that may have therapeutic applications in neurodegenerative disorders affecting central noradrenergic neurons, such as Alzheimer's, Parkinson's, and Huntington's diseases.

Animals↗
PubMed1995

Targeted disruption of Ca(2+)-calmodulin signaling in Drosophila growth cones leads to stalls in axon extension and errors in axon guidance.

Ca(2+)-calmodulin (CaM) function was selectively disrupted in a specific subset of growth cones in transgenic Drosophila embryos in which a specific enhancer element drives the expression of the kinesin motor domain fused to a CaM antagonist peptide (kinesin-antagonist or KA, which blocks CaM binding to target proteins) or CaM itself (kinesin-CaM or KC, which acts as a Ca(2+)-binding protein). In both KA and KC mutant embryos, specific growth cones exhibit dosage-dependent stalls in axon extension and errors in axon guidance, including both defects in fasciculation and abnormal crossings of the midline. These results demonstrate an in vivo function for Ca(2+)-CaM signaling in growth cone extension and guidance and suggest that Ca(2+)-CaM may in part regulate specific growth cone decisions, including when to defasciculate and whether or not to cross the midline.

Amino Acid Sequence↗
PubMed1994

Experimental seizures in the frog (Rana pipiens).

We investigated the effects of chemical convulsants in the leopard frog. Systemic kainic acid (5-20 mg/kg) caused limbic-like seizures, with staring, catatonia, fasciculations, and severe motor seizures, which were almost always lethal. Intracerebral electroencephalographic (EEG) recordings showed spike or spike-and-wave patterns at 6-8 Hz that decreased in frequency and increased in amplitude, maximal at an electrode in the midline olfactory/telencephalic (OLF-M) region. With time, an interictal pattern of 100-200 microV periodic spikes developed, followed by diffuse suppression of all brain activity. Seizures induced by pentylenetetrazole (150-450 mg/kg) and bicuculline (5-10 mg/kg) were characterized by the abrupt onset of motor activity, which continued intermittently for several hours, followed by recovery. EEG recordings in animals treated with pentylenetetrazole showed rhythmic spike-and-wave bursts at 1.5-3 Hz that were maximal at OLF-M. Recordings from frogs treated with bicuculline showed repetitive 3-6 Hz spike-and-wave discharges maximal at OLF-M that were nearly constant in amplitude and at times became continuous. Strychnine (1-5 mg/kg) caused reversible seizures characterized by tonic extensions of the extremities, that seemed to originate in the spinal cord. Frogs with recurrent seizures from systemic cis-diamminedichloroplatinum II showed 4-8 Hz rhythmic spike-and-wave activity that gradually slowed in frequency and increased in amplitude. Thus, the frog's reactivity to convulsive agents is similar to that of mammals.

Animals↗
PubMed1994

Capillary changes in angiokeratoma corporis diffusum Fabry.

We report on capillary changes on the Sulcus matricis unguis in a patient with an established angiokeratoma corporis diffusum Fabry. A fasciculated ramification of the capillary in 2-5 loops, which is not found in healthy individuals and that until now has solely been described in patients with vasculitis, was detected. The question now arises whether these are initial changes in the disease which later lead to manifested angiokeratomas. Further investigations will show if, and to what extent, these observations can contribute to further clarification of the developmental method of acroparaethesiae, which are part of the principal signs of Morbus Fabry.

Adult↗