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Titolo:
AGE-DEPENDENT PENETRANCE OF DISEASE IN A TRANSGENIC MOUSE MODEL OF FAMILIAL AMYOTROPHIC-LATERAL-SCLEROSIS
Autore:
CHIU AY; ZHAI P; DALCANTO MC; PETERS TM; KWON YW; PRATTIS SM; GURNEY ME;
Indirizzi:
CITY HOPE NATL MED CTR,BECKMAN RES INST,DIV NEUROSCI,1450 E DUARTE RDDUARTE CA 91010 NORTHWESTERN UNIV,SCH MED,DEPT CELL & MOLEC BIOL CHICAGO IL 60611 NORTHWESTERN UNIV,SCH MED,DEPT PATHOL CHICAGO IL 60611 NORTHWESTERN UNIV,SCH MED,CTR EXPTL ANIM RESOURCES CHICAGO IL 60611
Titolo Testata:
Molecular and cellular neurosciences
fascicolo: 4, volume: 6, anno: 1995,
pagine: 349 - 362
SICI:
1044-7431(1995)6:4<349:APODIA>2.0.ZU;2-8
Fonte:
ISI
Lingua:
ENG
Soggetto:
CU/ZN-SUPEROXIDE-DISMUTASE; PROGRAMMED CELL-DEATH; NEUROMUSCULAR-JUNCTION; DOWNS-SYNDROME; GROWTH-FACTOR; MICE; APOPTOSIS; PROTEIN; NERVE; ALS;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
46
Recensione:
Indirizzi per estratti:
Citazione:
A.Y. Chiu et al., "AGE-DEPENDENT PENETRANCE OF DISEASE IN A TRANSGENIC MOUSE MODEL OF FAMILIAL AMYOTROPHIC-LATERAL-SCLEROSIS", Molecular and cellular neurosciences, 6(4), 1995, pp. 349-362

Abstract

The mutation gly(93) --> ala of Cu,Zn superoxide dismutase (SOD) is found in patients with familiar amyotrophic lateral sclerosis and causes motor neuron disease when expressed in transgenic mice. The progression of clinical and pathological disease was studied in a line of micedesignated G1H. Clinical disease started at 91 +/- 14 days of age with fine shaking of the limbs, followed by paralysis and death by 136 +/- 7 days of age. Pathological changes begin by 37 days of age with vacuoles derived from swollen mitochondria accumulating in motor neurons. At the onset of clinical disease (90 days), significant death of somatic motor neurons innervating limb muscles has occurred; mice at end-stage disease (136 days) show up to 50% loss of cervical and lumbar motor neurons. However, neither thoracic nor cranial motor neurons show appreciable loss despite vacuolar changes. Autonomic motor neurons alsoare not affected. Mice that express wild-type human Cu,Zn son remain free of disease, indicating that mutations cause neuron loss by a gain-of-function. Thus, the age-dependent penetrance of motor neuron disease in this transgenic model is due to the gradual accumulation of pathological damage in select populations of cholinergic neurons.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 14/07/20 alle ore 19:12:35