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Differences in glutathione S-transferase pi expression in transgenic mice with symptoms of neurodegeneration

100%

Kaźmierczak B., Kuźma-Kozakiewicz M., Usarek E., Barańczyk-Kuźma A.

2011

vol. 58

issue 4

621-626

Glutathione S-transferase pi (GST pi) is an enzyme involved in cell protection against toxic electrophiles and products of oxidative stress. GST pi expression was studied in transgenic mice hybrids (B6-C3H) with symptoms of neurodegeneration harboring SOD1G93A (SOD1/+), Dync1h1 (Cra1/+) and double (Cra1/SOD1) mutations, at presymptomatic and symptomatic stages (age 70, 140, 365 days) using RT-PCR and Western blotting. The main changes in GST pi expression were observed in mice with the SODG93A mutation. In SOD1/+ and Cra1/SOD1 transgenics, with the exception of cerebellum, the changes in GST pi-mRNA accompanied those in GST pi protein. In brain cortex of both groups the expression was unchanged at the presymptomatic (age 70 days) but was lower at the symptomatic stage (age 140 days) and at both stages in hippocampus and spinal cord of SOD1/+ but not of Cra1/SOD1 mice compared to age-matched wild-type controls. In cerebellum of the presymptomatic and the symptomatic SOD1/+ mice and presymptomatic Cra1/SOD1 mice, the GST pi-mRNA was drastically elevated but the protein level remained unchanged. In Cra1/+ transgenics there were no changes in GST pi expression in any CNS region both on the mRNA and on the protein level. It can be concluded that the SOD1G93A but not the Dync1h1 mutation significantly decreases detoxification efficiency of GST pi in CNS, however the Dync1h1 mutation reduces the effects caused by the SOD1G93A mutation. Despite similarities in neurological symptoms, the differences in GST pi expression between SOD1/+ and Cra1/+ transgenics indicate a distinct pathogenic entity of these two conditions.

Stwardnienie boczne zanikowe – choroba neuronu ruchowego. Prezentacja przypadku

84%

Rubinowicz-Zasada M., Orczyk A., Orczyk M., Pasek J.

Pediatria i Medycyna Rodzinna

2015

vol. 11

issue 1

112-118

Amyotrophic lateral sclerosis, also known as Charcot’s disease and motor neuron disease, is a progressive neurodegenerative disease that causes muscle weakness, paralysis, and ultimately, respiratory failure. The aetiology and the pathogenesis of the syndrome remain unknown. Most people live 2–5 years after their first signs of the disease. There is no cure or effective treatment. We present a case of a female patient affected by progressing Charcot’s disease. On the Amyotrophic Lateral Sclerosis Functional Rating Scale – Revised (ALSFRS-R), the patient obtained 21 points. Atrophy and muscle spasm were very extended. Electromyography revealed features of coexisting denervation and reinnervation in the examined muscles. A growing number of Charcot’s disease cases require multidirectional actions to meet patient’s physical, emotional, and nutritional needs. Amyotrophic lateral sclerosis is an incurable disease. However, it is possible to relieve its symptoms by applying systematic physical rehabilitation.

Stwardnienie boczne zanikowe, znane jako choroba Charcota, dotyczy uszkodzenia neuronów ruchowych; jest to postępujące neurodegeneracyjne schorzenie powodujące patologiczne osłabienie mięśni, porażenia i niewydolność oddechową. Etiologia choroby oraz patogeneza wciąż pozostają nieznane. Większość chorych przeżywa tylko od 2 do 5 lat od momentu rozpoznania. Do tej pory nie ma skutecznej terapii. W pracy przedstawiono wyniki leczenia pacjentki z postępującą postacią stwardnienia bocznego zanikowego. Do oceny posłużono się skalą Functional Rating Scale, w której chora uzyskała 21 punktów. Zaniki mięśniowe oraz przykurcze były znacznie nasilone. W badaniu elektromiograficznym odnotowano zmiany unerwienia w ocenianych mięśniach. Wzrastająca liczba chorych wymaga wielokierunkowego działania, odnoszącego się do problemów fizycznych, emocjonalnych oraz potrzeb żywieniowych. Stwardnienie boczne zanikowe to choroba nieuleczalna, możliwe jest jednak łagodzenie jej objawów poprzez systematyczne prowadzenie rehabilitacji ruchowej.

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1 Acta Biochimica Polonica

1 Pediatria i Medycyna Rodzinna

1 2015

1 2011

1 Barańczyk-Kuźma A.

1 Kaźmierczak B.

1 Kuźma-Kozakiewicz M.

1 Orczyk A.

1 Orczyk M.

1 Pasek J.

1 Rubinowicz-Zasada M.

1 Usarek E.

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Differences in glutathione S-transferase pi expression in transgenic mice with symptoms of neurodegeneration

Stwardnienie boczne zanikowe – choroba neuronu ruchowego. Prezentacja przypadku