A single-chain Fv intrabody provides functional protection against the effects of mutant protein in an organotypic slice culture model of Huntington's disease.

Abstract	Huntington's disease (HD) is a progressive, hereditary, neurodegenerative disorder caused by an expanded polyglutamine tract in huntingtin protein, leading to misfolding and abnormal protein-protein interactions. Reducing the initial misfolding should lead to decreased pathogenesis. We show that malonate stress increases the number of dead or dying cells when organotypic slice cultures are transduced to express pathological-length huntingtin fragments. Co-transfected anti-HD single-chain Fv (sFv) intrabodies can reverse this HD-specific increase in malonate-induced morbidity.
Authors	Robert C Murphy, Anne Messer
Journal	Brain research. Molecular brain research (Brain Res Mol Brain Res) Vol. 121 Issue 1-2 Pg. 141-5 (Feb 05 2004) ISSN: 0169-328X [Print] Netherlands
PMID	14969746 (Publication Type: Comparative Study, Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References	Fv protein, human Htt protein, mouse Huntingtin Protein Immunoglobulin Variable Region Luminescent Proteins Lymphokines Malonates Nerve Tissue Proteins Nuclear Proteins Peptides Sialoglycoproteins Green Fluorescent Proteins polyglutamine Propidium malonic acid
Topics	Animals Animals, Newborn Biolistics (methods) Cell Aggregation (drug effects) Cell Count Cell Death (drug effects) Corpus Striatum (metabolism) Culture Techniques Disease Models, Animal Dose-Response Relationship, Drug Green Fluorescent Proteins Huntingtin Protein Huntington Disease (chemically induced, genetics, therapy) Immunoglobulin Variable Region (immunology, therapeutic use) Luminescent Proteins (metabolism) Lymphokines (therapeutic use) Malonates (toxicity) Mice Mice, Inbred BALB C Mutation Nerve Tissue Proteins (genetics, metabolism) Nuclear Proteins (genetics, metabolism) Peptides Propidium (metabolism) Sialoglycoproteins (therapeutic use) Time Factors Transfection

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.

Realize the full power of the drug-disease research graph!