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Literature DB >> 11042112

Aberrant phosphorylation of dentatorubral-pallidoluysian atrophy (DRPLA) protein complex in brain tissue.

Abstract

Dentatorubral-pallidoluysian atrophy (DRPLA) is caused by DRPLA protein which carries expansion of a glutamine repeat. Abnormal high-molecular-mass complex formation by DRPLA protein and its pathological ubiquitination comprise the disease processes in the brains of patients with DRPLA. In this study, DRPLA protein complex was isolated and shown to have pathologically stronger bond formation with DRPLA proteins in DRPLA brain tissue compared with control brain tissue. Immunochemical methods and an enzymic dephosphorylation technique were used to demonstrate that DRPLA protein complex is aberrantly phosphorylated in DRPLA brain tissue. Immunohistochemical studies show that both the ubiquitinated cytoplasmic inclusions and the nuclear membrane are aberrantly phosphorylated in DRPLA-affected neurons. This finding suggests that the nuclear membrane is another pathological focus of DRPLA neurodegeneration.

Entities: Chemical Disease Gene Species

Mesh：

Substances：

Year: 2000 PMID： 11042112 PMCID： PMC1221397

Source DB: PubMed Journal: Biochem J ISSN： 0264-6021 Impact factor: 3.857

30 in total

1. Unique characteristics of ubiquitin-bonded complex play a pathological role in dentatorubral-pallidoluysian atrophy.

Authors: I Yazawa
Journal: Biochem Biophys Res Commun Date: 1999-10-14 Impact factor: 3.575

2. Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17.

Authors: M Hong; V Zhukareva; V Vogelsberg-Ragaglia; Z Wszolek; L Reed; B I Miller; D H Geschwind; T D Bird; D McKeel; A Goate; J C Morris; K C Wilhelmsen; G D Schellenberg; J Q Trojanowski; V M Lee
Journal: Science Date: 1998-12-04 Impact factor: 47.728

3. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

Authors: M DiFiglia; E Sapp; K O Chase; S W Davies; G P Bates; J P Vonsattel; N Aronin
Journal: Science Date: 1997-09-26 Impact factor: 47.728

4. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

Authors: H L Paulson; M K Perez; Y Trottier; J Q Trojanowski; S H Subramony; S S Das; P Vig; J L Mandel; K H Fischbeck; R N Pittman
Journal: Neuron Date: 1997-08 Impact factor: 17.173

5. Expression of dentatorubral-pallidoluysian atrophy (DRPLA) proteins in patients.

Authors: I Yazawa; N Nukina; J Goto; H Kurisaki; A Hebisawa; I Kanazawa
Journal: Neurosci Lett Date: 1997-03-28 Impact factor: 3.046

6. Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats.

Authors: G Imbert; F Saudou; G Yvert; D Devys; Y Trottier; J M Garnier; C Weber; J L Mandel; G Cancel; N Abbas; A Dürr; O Didierjean; G Stevanin; Y Agid; A Brice
Journal: Nat Genet Date: 1996-11 Impact factor: 38.330

7. Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel.

Authors: O Zhuchenko; J Bailey; P Bonnen; T Ashizawa; D W Stockton; C Amos; W B Dobyns; S H Subramony; H Y Zoghbi; C C Lee
Journal: Nat Genet Date: 1997-01 Impact factor: 38.330

8. Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion.

Authors: G David; N Abbas; G Stevanin; A Dürr; G Yvert; G Cancel; C Weber; G Imbert; F Saudou; E Antoniou; H Drabkin; R Gemmill; P Giunti; A Benomar; N Wood; M Ruberg; Y Agid; J L Mandel; A Brice
Journal: Nat Genet Date: 1997-09 Impact factor: 38.330

9. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

Authors: S W Davies; M Turmaine; B A Cozens; M DiFiglia; A H Sharp; C A Ross; E Scherzinger; E E Wanker; L Mangiarini; G P Bates
Journal: Cell Date: 1997-08-08 Impact factor: 41.582

Aberrant phosphorylation of dentatorubral-pallidoluysian atrophy (DRPLA) protein complex in brain tissue.

1. Unique characteristics of ubiquitin-bonded complex play a pathological role in dentatorubral-pallidoluysian atrophy.

2. Mutation-specific functional impairments in distinct tau isoforms of hereditary FTDP-17.

3. Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

4. Intranuclear inclusions of expanded polyglutamine protein in spinocerebellar ataxia type 3.

5. Expression of dentatorubral-pallidoluysian atrophy (DRPLA) proteins in patients.

6. Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats.

7. Autosomal dominant cerebellar ataxia (SCA6) associated with small polyglutamine expansions in the alpha 1A-voltage-dependent calcium channel.

8. Cloning of the SCA7 gene reveals a highly unstable CAG repeat expansion.

9. Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

10. Different complex formations of dentatorubral-pallidoluysian atrophy (DRPLA) protein in human and rat neurons.

1. Repeat-associated non-ATG (RAN) translation.

Review 2. Nuclear transport, oxidative stress, and neurodegeneration.

Review 3. New developments in RAN translation: insights from multiple diseases.

Review 4. Exploring the Role of Posttranslational Modifications in Spinal and Bulbar Muscular Atrophy.