Are Lymphoblastoid Cell Lines Suitable to Study Increased SMN Gene Expression by Histone Deacetylase Inhibitors?

Didem Dayangaç ERDEN; Haluk TOPALOĞLU; Hayat Erdem YURTER

Article Details

Are Lymphoblastoid Cell Lines Suitable to Study Increased SMN Gene Expression by Histone Deacetylase Inhibitors?

Authors:

Didem Dayangaç ERDEN | Haluk TOPALOĞLU | Hayat Erdem YURTER

Publisher:

TUBITAK

Journal:

Turkish Journal of Medical Sciences 2008 Vol.38 Issue 1, pp.7-12

Document Type:

Article

Publication Date:

12/2007

Document Language:

Turkish

Document ID (Org No.):

2000755875

Frequency:

Bimonthly

eISSN:

1303-6165

pISSN:

1300-0144

Keywords:

Abstract:

 

Aim: In this preliminary study, we aimed to analyze the effect of sodium butyrate (NB) and phenylbutyrate (PB), belonging to the class of histone deacetylase (HDAC) inhibitors, on lymphoblastoid cell lines established from spinal muscular atrophy (SMA) patients.   Materials and Methods: Lymphoblastoid cell lines were established from one type I and two type III SMA patients. Following treatment of the cell lines by HDAC inhibitors, the levels of survival motor neuron 2 (SMN2) gene full length (fl-SMN2) transcripts were assessed by both standard and real time RT-PCR. To determine the levels of SMN protein, Western blot analysis was performed.   Results: NB and PB, which are known to be effective in SMA fibroblasts, did not increase the levels of fl-SMN2 transcripts or protein in lymphoblastoid cell lines.   Conclusions: Epstein-Barr virus (EBV)-transformed lymphoblastoid cell lines may not be suitable for studying the effect of at least some HDAC inhibitors on SMN2 gene expression.

 

Aim: In this preliminary study, we aimed to analyze the effect of sodium butyrate (NB) and phenylbutyrate (PB), belonging to the class of histone deacetylase (HDAC) inhibitors, on lymphoblastoid cell lines established from spinal muscular atrophy (SMA) patients.   Materials and Methods: Lymphoblastoid cell lines were established from one type I and two type III SMA patients. Following treatment of the cell lines by HDAC inhibitors, the levels of survival motor neuron 2 (SMN2) gene full length (fl-SMN2) transcripts were assessed by both standard and real time RT-PCR. To determine the levels of SMN protein, Western blot analysis was performed.   Results: NB and PB, which are known to be effective in SMA fibroblasts, did not increase the levels of fl-SMN2 transcripts or protein in lymphoblastoid cell lines.   Conclusions: Epstein-Barr virus (EBV)-transformed lymphoblastoid cell lines may not be suitable for studying the effect of at least some HDAC inhibitors on SMN2 gene expression.

Article Details

Are Lymphoblastoid Cell Lines Suitable to Study Increased SMN Gene Expression by Histone Deacetylase Inhibitors?

Authors:

Didem Dayangaç ERDEN | Haluk TOPALOĞLU | Hayat Erdem YURTER

Publisher:

TUBITAK

Journal:

Turkish Journal of Medical Sciences 2008 Vol.38 Issue 1, pp.7-12

Document Type:

Article

Publication Date:

12/2007

Document Language:

Turkish

Document ID (Org No.):

2000755875

Frequency:

Bimonthly

eISSN:

1303-6165

pISSN:

1300-0144

Spinal muscular atrophy

survival motor neuron gene

histone deacetylase inhibitors

treatment

Issues

Article Details

Are Lymphoblastoid Cell Lines Suitable to Study Increased SMN Gene Expression by Histone Deacetylase Inhibitors?

Authors: Didem Dayangaç ERDEN | Haluk TOPALOĞLU | Hayat Erdem YURTER

Publisher: TUBITAK

Journal: Turkish Journal of Medical Sciences 2008 Vol.38 Issue 1, pp.7-12

Document Type: Article

Publication Date: 12/2007

Document Language: Turkish

Document ID (Org No.): 2000755875

Frequency: Bimonthly

eISSN: 1303-6165

pISSN: 1300-0144

Spinal muscular atrophy

survival motor neuron gene

histone deacetylase inhibitors

treatment

Issues

Authors:

Didem Dayangaç ERDEN | Haluk TOPALOĞLU | Hayat Erdem YURTER

Publisher:

TUBITAK

Journal:

Turkish Journal of Medical Sciences 2008 Vol.38 Issue 1, pp.7-12

Document Type:

Article

Publication Date:

12/2007

Document Language:

Turkish

Document ID (Org No.):

2000755875

Frequency:

Bimonthly

eISSN:

1303-6165

pISSN:

1300-0144