Ovine Arthrogryposis and Central Nervous System Malformations Associated with in utero Cache Valley Virus Infection: Spontaneous Disease

1989 ◽  
Vol 26 (1) ◽  
pp. 33-39 ◽  
Author(s):  
J. F. Edwards ◽  
C. W. Livingston ◽  
S. I. Chung ◽  
E. C. Collisson
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Motor Neurons ◽  
Spinal Column ◽  
In Utero ◽  
Cache Valley Virus ◽  
Cache Valley ◽  
Seroepidemiologic Studies ◽  
Heart Blood

Gross appearance and histologic lesions seen in 15 newborn lambs in an outbreak of congenital arthrogryposis with hydrocephalus or hydranencephaly (CAH) in Texas are described. Severe arthrogryposis with skeletal muscle hypoplasia was seen in limbs of affected lambs. Spinal column deformities were also present. Multiple central nervous system (CNS) malformations were present in CAH lambs including micrencephaly, cerebellar hypoplasia, micromelia, hydrocephalus, hydranencephaly, and porencephaly. Histologic lesions consisted primarily of areas of necrosis and loss of the paraventricular neuropil and motor neurons in the CNS and a resolving myositis with poorly developed myotubular myocytes in skeletal muscle. Seroepidemiologic studies on the flock and serologic data from heart blood taken from the stillborn affected lambs indicated that the outbreak was due to in utero infection with Cache Valley Virus.

Hippocampal glutamine synthetase: insensitivity to glucocorticoids and stress

1990 ◽  
Vol 258 (5) ◽  
pp. E894-E897 ◽  
Author(s):  
G. C. Tombaugh ◽  
R. M. Sapolsky
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Glutamine Synthetase ◽  
Glutamate Metabolism ◽  
Glutamatergic Synapses ◽  
Adult Rats ◽  
Glucocorticoid Induction ◽  
The Central Nervous System ◽  
The Brain

Glucocorticoids enhance the neurotoxic potential of several insults to the rat hippocampus that involve overactivation of glutamatergic synapses. These hormones also stimulate the synthesis of glutamine synthetase (GS) in peripheral tissue. Because this enzyme helps regulate glutamate metabolism in the central nervous system, glucocorticoid induction of GS in the brain may underlie the observed synergy. We have measured GS activity in the hippocampus and skeletal muscle (plantaris) of adult rats after bilateral adrenalectomy (ADX), corticosterone (Cort) replacement, or stress. No significant changes in GS were observed in hippocampal tissue, whereas muscle GS was significantly elevated after Cort treatment or stress and was reduced after ADX. These results suggest that Cort-induced shifts in GS activity probably do not explain Cort neurotoxicity, although the stress-induced rise in muscle GS may be relevant to certain types of myopathy.

Primitive Neuroectodermal Tumor of Cerebrum With Adipose Tissue

2001 ◽  
Vol 125 (2) ◽  
pp. 264-266
Author(s):  
Satish Krishnamurthy ◽  
Stephen Kent Powers ◽  
Javad Towfighi
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Adipose Tissue ◽  
Smooth Muscle ◽  
Primitive Neuroectodermal Tumor ◽  
Primitive Neuroectodermal Tumors ◽  
Mature Adipose Tissue ◽  
Neuroectodermal Tumors ◽  
The Central Nervous System

Abstract Primitive neuroectodermal tumors (PNETs) of the central nervous system are uncommon embryonal neoplasms, rarely occurring in adults. Differentiation into specific mesenchymal tissues, such as cartilage, bone, skeletal muscle, smooth muscle, or adipose tissue, is rare. We report a case of a 51-year-old woman with a PNET of cerebrum that showed extensive mature adipose tissue differentiation. This is the second case, to our knowledge, of PNET of cerebrum with adipose tissue elements that has been described.

The role of globins in cardiovascular physiology

2021 ◽  
Author(s):  
T.C. Steven Keller ◽  
Christophe Lechauve ◽  
Alexander S Keller ◽  
Steven Brooks ◽  
Mitchell J Weiss ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Smooth Muscle ◽  
Vascular Smooth Muscle ◽  
Smooth Muscle Cells ◽  
Muscle Cells ◽  
Cell Types ◽  
Specific Cell ◽  
In Cells

Globin proteins exist in every cell type of the vasculature, from erythrocytes to endothelial cells, vascular smooth muscle cells, and peripheral nerve cells. Many globin subtypes are also expressed in muscle tissues (including cardiac and skeletal muscle), in other organ-specific cell types, and in cells of the central nervous system. The ability of each of these globins to interact with molecular oxygen (O2) and nitric oxide (NO) is preserved across these contexts. Endothelial α-globin is an example of extra-erythrocytic globin expression. Other globins, including myoglobin, cytoglobin, and neuroglobin are observed in other vascular tissues. Myoglobin is observed primarily in skeletal muscle and smooth muscle cells surrounding the aorta or other large arteries. Cytoglobin is found in vascular smooth muscle but can also be expressed in non-vascular cell types, especially in oxidative stress conditions after ischemic insult. Neuroglobin was first observed in neuronal cells, and its expression appears to be restricted mainly to the central and peripheral nervous systems. Brain and central nervous system neurons expressing neuroglobin are positioned close to many arteries within the brain parenchyma and can control smooth muscle contraction and, thus, tissue perfusion and vascular reactivity. Overall, reactions between NO and globin heme-iron contribute to vascular homeostasis by regulating vasodilatory NO signals and scaveging reactive species in cells of the mammalian vascular system. Here, we discuss how globin proteins affect vascular physiology with a focus on NO biology, and offer perspectives for future study of these functions.

Skin and Bones—and Muscle Too

Bioprinting ◽  
2021 ◽  
pp. 98-118
Author(s):  
Kenneth Douglas
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Cell Membrane ◽  
Motor Neuron ◽  
Neuromuscular Junctions ◽  
Skeletal Muscle Cell ◽  
The Body ◽  
The Central Nervous System ◽  
Judicious Choice

Abstract: This chapter recounts bioprinting studies of skin, bone, skeletal muscle, and neuromuscular junctions. The chapter begins with a study of bioprinted skin designed to enable the creation of skin with a uniform pigmentation. The chapter relates two very different approaches to bioprinted bone: a synthetic bone called hyperelastic bone and a strategy that prints cartilage precursors to bone and then induces the conversion of the cartilage to bone by judicious choice of bioinks. Muscles move bone, and the chapter discusses an investigation of bioprinted skeletal muscle. Finally, the chapter considers an attempt to bioprint a neuromuscular junction, a synapse—a minute gap—of about 20 billionths of a meter between a motor neuron and the cell membrane of a skeletal muscle cell. A motor neuron is a nerve in the central nervous system that sends signals to the muscles of the body.

Degeneration of skeletal muscle, peripheral nerves, and the central nervous system in transgenic mice overexpressing wild-type prion proteins

Cell ◽  
1994 ◽  
Vol 76 (1) ◽  
pp. 117-129 ◽  
Author(s):  
David Westaway ◽  
Stephen J. DeArmond ◽  
Juliana Cayetano-Canlas ◽  
Darlene Groth ◽  
Dallas Foster ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Central Nervous System ◽  
Nervous System ◽  
Transgenic Mice ◽  
Peripheral Nerves ◽  
Prion Proteins ◽  
Wild Type ◽  
The Central Nervous System

scholarly journals Loss of the nucleoporin Aladin in central nervous system and fibroblasts of Allgrove Syndrome

2019 ◽  
Vol 28 (23) ◽  
pp. 3921-3927 ◽  
Author(s):  
Giacomo Bitetto ◽  
Dario Ronchi ◽  
Sara Bonato ◽  
Alessandra Pittaro ◽  
Giacomo Monzio Compagnoni ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Purkinje Cells ◽  
Motor Neurons ◽  
Nuclear Membrane ◽  
Nucleocytoplasmic Transport ◽  
Neurological Involvement ◽  
Nuclear Pore ◽  
Striatal Neurons ◽  
Allgrove Syndrome

Abstract Allgrove syndrome (AS) is a rare disease with broad neurological involvement. Neurodegeneration can affect spinal motor neurons, Purkinje cells, striatal neurons and the autonomic system. The mechanisms that lead to neuronal loss are still unclear. Recessive mutations in the AAAS gene affect the encoded protein Aladin, which would normally localize to the cytoplasmic face of the nuclear membrane as part of the nuclear pore complex (NPC). While the NPC is known to be a key factor for nucleocytoplasmic transport, the precise role of Aladin has not been elucidated yet. Here, we explored the consequences of the homozygous AAAS mutation c.464G>A (p.R155H) in central nervous system tissues and fibroblasts of a novel AS patient presenting motor neuron disease, cerebellar ataxia and autonomic dysfunction. Neuropathological analyses showed severe loss of motor neurons and Purkinje cells, with significant reduction in the perinuclear expression of Aladin. A reduced amount of protein was detected in the nuclear membrane fraction of the patient’s brain. RNA analysis revealed a significant reduction of the transcript AAAS-1, while the AAAS-2 transcript was upregulated in fibroblasts. To our knowledge, this is the first study to demonstrate the effects of AAAS mutations in the human central nervous system.

Dopamine modulation of gill reflex behavior in Aplysia

1979 ◽  
Vol 57 (3) ◽  
pp. 329-332 ◽  
Author(s):  
Peter Ruben ◽  
Ken Lukowiak
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Motor Neuron ◽  
Peripheral Nervous System ◽  
Motor Neurons ◽  
Withdrawal Reflex ◽  
Dopaminergic Pathway ◽  
The Central Nervous System ◽  
Dopamine Modulation

We have studied the effects of dopamine on the gill withdrawal reflex evoked by tactile siphon stimulation in the margine mollusc Aplysia. Physiological concentrations of dopamine (diluted in seawater) were perfused through the gill during siphon stimulation series. The amplitude of the reflex was potentiated by dopamine and habituation of the reflex was prevented. This occurred with no change in the activity evoked in central motor neurons. These results lead us to conclude that the dopaminergic motor neuron L9 is modulating habituation in the periphery and that the central nervous system facilitatory control of the peripheral nervous system may act via a dopaminergic pathway.

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