Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:1.9.3.1 (cytochrome oxidase)
 8,822 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

NFH-LacZ transgenic mice are characterized by an early accumulation of the neurofilament cytoskeleton in the cell bodies of neurons with age-associated abnormalities of motor neurons and cerebellar Purkinje cells. In comparison to normal littermate controls, irrespective of age (3 and 12-20 months), NFH-LacZ transgenic mice had a lower number of rears in an open field, deficiencies in some motor-coordination tests, and a higher number of quadrant entries and escape latencies while swimming toward a visible platform. Decreased cytochrome oxidase activity in the lateral reticular nucleus of NFH-LacZ mice was associated with poor performance in two motor coordination tests. Lower metabolic activity in the lateral reticular nucleus may be secondary to previously described cerebellar abnormalities, leading to deficient motor control. The dramatic cytoskeletal perturbation characterizing NFH-LacZ mice affects only selective neuronal populations and results in selective behavioral deficits, which can be correlated with regional brain metabolic activity.
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PMID:Motor coordination and spatial orientation are affected by neurofilament maldistribution: correlations with regional brain activity of cytochrome oxidase. 1036 45

Expression of the NFH-LacZ fusion protein in transgenic mice causes an early accumulation of neurofilament proteins in the cell bodies of neurons, as well as a reduction of motor neuron axonal caliber and Purkinje cell number in the cerebellum. Young (3 month old) and older (12-20 months) NFH-LacZ transgenic mice were compared to normal controls for regional brain metabolism, as assessed by cytochrome oxidase (CO) activity. Irrespective of age, CO activity was reduced in three cerebellar-related regions of NFH-LacZ transgenic mice: (1) the lateral reticular nucleus, (2) the parvicellular red nucleus, and (3) the superior colliculus, possibly as a secondary consequence of cerebellar Purkinje cell histopathology. Aged NFH-LacZ mice had lower CO activity relative to either age-matched controls or young transgenic mice in the following regions: the motor nucleus of the vagus nerve, the trapezoid nucleus, the subiculum, the motor cortex, the superior olive, and the lateral dorsal thalamus. These results indicate regional and age-selective deficits of brain metabolism in a transgenic model with neurofilament maldistribution.
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PMID:NFH-LacZ transgenic mice: regional brain activity of cytochrome oxidase. 1242 97

Neuronal intermediate filaments consist of the NFL subunit linked with NFM and NFH, and their alterations have been proposed as a pathogenesic cause in motor neuron diseases. Depletion of the Nefl gene in mice mimicks the reduced NFL mRNA levels seen in amyotrophic lateral sclerosis and causes perikaryal accumulation of neurofilament proteins and axonal hypotrophy in motoneurons. NFL -/- mice were evaluated for regional brain metabolism by means of quantitative histochemical estimation of cytochrome oxidase (COx) activity. The NFL null mice displayed enzymatic activity alterations in numerous hindbrain regions, mainly the cerebellum, connected regions of the brainstem (red nucleus, vestibular nuclei, and reticular formation), and cranial nerve nuclei. All of the affected regions presented elevated COx activity, except for the Purkinje cells of the cerebellum and the magnocellular red nucleus, where enzymatic activity was lower. NFL-disrupted mice displayed functional alterations in brainstem sensorimotor regions affected in amyotrophic lateral sclerosis.
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PMID:Mice with the deleted neurofilament of low-molecular-weight (Nefl) gene: 1. Effects on regional brain metabolism. 1574 62