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Induced pluripotent stem cells in modeling and cell-based therapy of amyotrophic lateral sclerosis

100%

Csobonyeiova M., Polak S., Nicodemou A., Danisovic L.

Journal of Physiology and Pharmacology

2017

tom 68

nr 5

CAG repeat polymorphism in the androgen receptor [AR] gene of SBMA patients and a control group

84%

Sulek A., Hoffman-Zacharska D., Krysa W., Szirkowiec W., Fidzianska E., Zaremba J.

Journal of Applied Genetics

2005

tom 46

nr 2

Spinobulbar muscular atrophy (SBMA) is an X-linked form of motor neuron disease characterized by progressive atrophy of the muscles, dysphagia, dysarthria and mild androgen insensitivity. SBMA is caused by CAG repeat expansion in the androgen receptor gene. CAG repeat polymorphism was analysed in a Polish control group (n = 150) and patients suspected of SBMA (n = 60). Normal and abnormal ranges of CAG repeats were established in the control group and in 21 patients whose clinical diagnosis of SBMA was molecularly confirmed. The ranges are similar to those reported for other populations.

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

67%

Kazmierczak B., Kuzma-Kozakiewicz M., Usarek E., Baranczyk-Kuzma A.

Acta Biochimica Polonica

2011

tom 58

nr 4

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.

Ograniczanie wyników

1 Acta Biochimica Polonica

1 Journal of Applied Genetics

1 Journal of Physiology and Pharmacology

1 Baranczyk-Kuzma A.

1 Csobonyeiova M.

1 Danisovic L.

1 Fidzianska E.

1 Hoffman-Zacharska D.

1 Kazmierczak B.

1 Krysa W.

1 Kuzma-Kozakiewicz M.

1 Nicodemou A.

1 Polak S.

1 Sulek A.

1 Szirkowiec W.

1 Usarek E.

1 Zaremba J.

1 2017

1 2011

1 2005

Wyniki wyszukiwania

Induced pluripotent stem cells in modeling and cell-based therapy of amyotrophic lateral sclerosis

CAG repeat polymorphism in the androgen receptor [AR] gene of SBMA patients and a control group

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