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Titolo:
Molecular factors underlying selective vulnerability of motor neurons to neurodegeneration in amyotrophic lateral sclerosis
Autore:
Shaw, PJ; Eggett, CJ;
Indirizzi:
Royal Victoria Infirm, Dept Neurol, Newcastle Upon Tyne NE1 4LP, Tyne & Wear, England Royal Victoria Infirm Newcastle Upon Tyne Tyne & Wear England NE1 4LP and
Titolo Testata:
JOURNAL OF NEUROLOGY
, volume: 247, anno: 2000, supplemento:, 1
pagine: 17 - 27
SICI:
0340-5354(200003)247:<17:MFUSVO>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
CU/ZN SUPEROXIDE-DISMUTASE; GLUTAMATE-RECEPTOR SUBUNITS; CALCIUM-BINDING PROTEINS; HUMAN SPINAL-CORD; TRANSGENIC MICE; NEUROFILAMENT SUBUNIT; PHOSPHORYLATED NEUROFILAMENTS; NEUROLOGICAL DISORDERS; TRANSPORTER EAAT2; ENERGY-METABOLISM;
Keywords:
motor neurons; selective vulnerability; amyotrophic lateral sclerosis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
95
Recensione:
Indirizzi per estratti:
Indirizzo: Shaw, PJ Royal Victoria Infirm, Dept Neurol, Ward 11, Newcastle Upon Tyne NE1 4LP, Tyne & Wear, England Royal Victoria Infirm Ward 11 Newcastle Upon Tyne Tyne & Wear England NE1 4LP
Citazione:
P.J. Shaw e C.J. Eggett, "Molecular factors underlying selective vulnerability of motor neurons to neurodegeneration in amyotrophic lateral sclerosis", J NEUROL, 247, 2000, pp. 17-27

Abstract

Current research evidence suggests that genetic factors, oxidative stress and glutamatergic toxicity, with damage to critical target proteins and organelles, may be important contributory factors to motor neuron injury in amyotrophic lateral sclerosis (ALS). Various molecular and neurochemical features of human motor neurons may render this cell group differentially vulnerable to such insults. Motor neurons are large cells with long axonal processes which lead to requirements for a high level of mitochondrial activity and a high neurofilament content compared to other neuronal groups. The lack of calcium buffering proteins parvalbumin and calbindin D28k and the low expression of the GluR2 AMPA receptor subunit may render human motor neurons particularly vulnerable to calcium toxicity following glutamate receptor activation. Motor neurons also have a high perisomatic expression of the glutamate transporter protein EAAT2 and a very high expression of the cytosolic free radical scavenging enzyme Cu/Zn superoxide dismutase (SOD1) which may render this cell group vulnerable in the face of genetic or post-translational alterations interfering with the function of these proteins. More detailed characterisation of the molecular features of human motor neurons inthe future may allow the strategic development of better neuroprotective therapies for the benefit of patients afflicted by ALS.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 20/01/20 alle ore 05:37:57