NDLI: Proteomic analysis of a drosophila IBMPFD model reveals potential pathogenic mechanisms

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Proteoglycan sequence

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Proteomic analysis of a drosophila IBMPFD model reveals potential pathogenic mechanisms

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Proteomic analysis of a drosophila IBMPFD model reveals potential pathogenic mechanisms

Content Provider	Royal Society of Chemistry (RSC)
Author	Huang, Shun-Hong Chan, Hsin-Tzu Lee, Hsiao-Yun Sang, Tzu-kang Chan, Hong-Lin Lyu, Ping-Chiang Lee, Tian-Ren
Copyright Year	2012
Abstract	IBMPFD, Inclusion body myopathy associated with Paget's disease of bone and frontotemporal dementia, is a hereditary degenerative disorder due to single missense mutations in VCP (Valosin-Containing Protein). The mechanisms of how mutations of VCP lead to IBMPFD remain mysterious. Here we utilize two-dimensional difference gel electrophoresis (2D-DIGE) combined with mass spectrometry to study the IBMPFD disorder at the protein level. With this set-up, we are able to employ comparative proteomics to analyze IBMPFD disease using Drosophila melanogaster as our disease model organism. Head proteome of transgenic D. melanogaster expressing wild type VCP is compared, respectively, with the head proteome of transgenic mutant type VCPs that correspond to human IBMPFD disease alleles (TER94A229E, TER94R188Q, and TER94R152H). Of all the proteins identified, a significant fraction of proteins altered in TER94A229E and TER94R188Q mutants belong to the same functional categories, i.e. apoptosis and metabolism. Among these, Drosophila transferrin is observed to be significantly up-regulated in mutant flies expressing TER94A229E. A knock-down experiment suggests that fly transferrin might be a potential modifier in IBMPFD disease. The molecular analysis of IBMPFD disease may benefit from the proteomics approach which combines the advantages of high throughput analysis and the focus on protein levels.
Starting Page	1730
Ending Page	1741
Page Count	12
File Format	HTM / HTML PDF
ISSN	1742206X
Volume Number	8
Issue Number	6
Journal	Molecular BioSystems
DOI	10.1039/c2mb25037c
Language	English
Publisher	Royal Society of Chemistry
Access Restriction	Open
Subject Keyword	VCP IBMPFD Transferrin Proteome Metabolism Model organism Difference gel electrophoresis Protein Paget Proteomics Bone Mutation Frontotemporal dementia Mass spectrometry Drosophila melanogaster Myopathy Apoptosis
Content Type	Text
Resource Type	Article
Subject	Molecular Biology Biotechnology

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Inclusion Body Myopathy with Paget Disease of Bone and/or Frontotemporal Dementia

A Brazilian family with hereditary inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia

A progressive translational mouse model of human valosin-containing protein disease: The VCP$ ^{R155H/+}$ mouse

Generation, Analyzing and in-vivo Drug Treatment of Drosophila Models with IBMPFD.

A Brazilian family with hereditary inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia

A Brazilian family with hereditary inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia

Neurological and dysmorphic features in a family with brachial plexus neuropathy

A clinicopathologic study of malignancy in VCP-associated multisystem proteinopathy

Global Gene Profiling of VCP-associated Inclusion Body Myopathy

Proteomic analysis of a drosophila IBMPFD model reveals potential pathogenic mechanisms

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