Polyglutamine residues from Machado-Joseph disease gene enhance formation of aggregates of GST-polyglutamine fusion protein in E. coli

Hyangshuk Rhim, Kyoung Sook Bok, Mi Jeong Chang, In Kyung Kim, Sung Sup Park, Seong Man Kang

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

Several neurodegenerative diseases such as Huntington's disease, dentatorubralpallidoluysian atrophy, spinobulbar muscular atrophy, Machado- Joseph disease, and spinocerebellar ataxias type 1 are associated with the aggregation of expanded glutamine repeats within their proteins. Generally, in clinically affected individuals, the expansion of the polyglutamine sequences is beyond 40 residues. To address the length of polyglutamine that forms aggregation, we have constructed plasmids encoding glutathione S- transferase (GST) Machado-Joseph disease gene fusion proteins containing polyglutamine and investigated the formation of aggregates in E. coli. Surprisingly, even (Gln)8 in the normal range as well as (Gln)65 in the pathogenic range enhanced the formation of insoluble protein aggregates, whereas (Ser)8 and (Ala)8 did not form aggregates. Our results indicate that the formation of protein aggregates in GST-polyglutamine proteins is specifically mediated by the polyglutamine repeat sequence within their protein structure. Our study may contribute to the understanding of the molecular mechanism of the formation of protein aggregates in neurodegenerative disorders and the development of preventative strategies.

Original languageEnglish
Pages (from-to)663-668
Number of pages6
JournalJournal of Microbiology and Biotechnology
Volume8
Issue number6
Publication statusPublished - 1998 Dec 1

Fingerprint

Machado-Joseph Disease
Escherichia coli Proteins
Glutathione Transferase
Escherichia coli
Fusion reactions
Genes
Proteins
Neurodegenerative Diseases
Agglomeration
Atrophic Muscular Disorders
Neurodegenerative diseases
Spinocerebellar Ataxias
Gene Fusion
Huntington Disease
Glutamine
Atrophy
Reference Values
Plasmids
polyglutamine
Glutathione

Keywords

  • Aggregates
  • GST-fusion proteins
  • MJD gene
  • Polyglutamine

ASJC Scopus subject areas

  • Biotechnology
  • Applied Microbiology and Biotechnology
  • Microbiology
  • Bioengineering

Cite this

Polyglutamine residues from Machado-Joseph disease gene enhance formation of aggregates of GST-polyglutamine fusion protein in E. coli. / Rhim, Hyangshuk; Bok, Kyoung Sook; Chang, Mi Jeong; Kim, In Kyung; Park, Sung Sup; Kang, Seong Man.

In: Journal of Microbiology and Biotechnology, Vol. 8, No. 6, 01.12.1998, p. 663-668.

Research output: Contribution to journalArticle

Rhim, Hyangshuk ; Bok, Kyoung Sook ; Chang, Mi Jeong ; Kim, In Kyung ; Park, Sung Sup ; Kang, Seong Man. / Polyglutamine residues from Machado-Joseph disease gene enhance formation of aggregates of GST-polyglutamine fusion protein in E. coli. In: Journal of Microbiology and Biotechnology. 1998 ; Vol. 8, No. 6. pp. 663-668.
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