Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UNIPROT:P20366 (substance P)
 21,176 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Neuropeptide and neurotransmitter plasticity has been demonstrated in the central nervous system. Modifications of their synthesis occur following receptor blockade or deafferentiation by surgical lesions. This concept should provid answers to some remaining open questions in human pathology especially in degenerative diseases of the basal ganglia. In a severely atrophied striatum we observed a selective increase in the number of detectable striatal substance P and met-enkephalin neurones which exhibited a striking increase in the intensity of labelling. This increase, instead of the well established reduction of substance P and enkephalins in the atrophied striatum of Huntington's disease, could explain the absence of choreoathetosis which was replaced by rigidity and bradykinesia in the patient. The absence of choreoathetosis, despite severe striatal atrophy, is described in several basal ganglia diseases and could also be related to neurotransmitter or neuropeptide plasticity rather than due to the primary lesion.
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PMID:Does the absence of clinical expression of choreoathetosis, despite severe striatal atrophy, correlate with plasticity of neuropeptide synthesis? 175 57

Huntington's Disease (HD) is a progressive degenerative disorder of the central nervous system inherited as an autosomal dominant trait. Clinically, the disorder is characterized by choreoathetosis (with age of onset typically in the late thirties or early forties) and neuropsychiatric disturbance. The striatum is particularly vulnerable to the degenerative disease process, with selective loss of medium spiny neurons and decreased levels of associated neurotransmitters, including substance P. GABA, met-enkephalin and dynorphin. Although the underlying pathophysiology is unknown, recent theories concerning pathogenesis have involved mitochondrial abnormalities and excitotoxin-mediated damage. The gene for HD has recently been discovered and characterized as an unstable CAG trinucleotide repeat sequence on the short arm of chromosome 4 (now known as IT15). The direct test now available for the HD gene has facilitated disease diagnosis, particularly for those with unclear family history or chorea of uncertain origin; presymptomatic testing is also available. Management of affected individuals is unsatisfactory as only symptomatic control is available. However, as the effect of the genetic abnormality may soon be known, specific treatment of the disorder may become available in the near future.
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PMID:Huntington's disease: recent advances in diagnosis and management. 775 74

In a severely demented patient, with a family history of dementia, a severe striatal atrophy was observed. Neither this patient, nor his relatives, had choreoathetosis but he displayed rigidity and bradykinesia. A selective increase of the number of detectable striatal substance P and met-enkephalin neurones was found. These neurones also exhibited a striking increase of the intensity of these peptides' immunoreactivities. Simultaneously, the dense networks of substance P and met-enkephalin nerve fibres were well preserved in the globus pallidus and the substantia nigra. The absence of choreoathetosis, despite severe striatal atrophy, is described in several basal ganglia diseases. Our results are in contrast with the well established reductions of substance P and enkephalins in the atrophied striatum as well as in the globus pallidus and the substantia nigra reported in classical cases of Huntington's disease expressing choreoathetosis.
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PMID:Increase of substance P and met-enkephalin in a severely atrophied striatum without clinical expression of chorea. 2050 15