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Antibodies and Intrabodies Against Huntingtin: Production and Screening of Monoclonals and Single-Chain Recombinant Forms

Khoshnan, Ali and Ou, Susan and Ko, Jan and Patterson, Paul H. (2013) Antibodies and Intrabodies Against Huntingtin: Production and Screening of Monoclonals and Single-Chain Recombinant Forms. In: Trinucleotide Repeat Protocols. Methods in Molecular Biology. No.1010. Humana Press , New York, pp. 231-251. ISBN 978-1-62703-410-4.

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Antibodies can be extremely useful tools for the field of triplet repeats diseases. These reagents are important for localizing proteins in tissues and they can be used in the isolation and characterization of the components of protein complexes. In the context of huntingtin (Htt), antibodies can distinguish Htt with normal or an expanded polyglutamine (polyQ) repeats, and they can identify distinct conformations of Htt. Htt is the protein that, when mutated to contain an expanded polyQ motif, causes Huntington’s disease (HD). Our group has produced monoclonal and recombinant single-chain antibodies (intrabodies) that can be used for these purposes and to perturb the function of Htt in living cells. Studies with anti-Htt intrabodies have led to identification of novel pathogenic epitopes. Moreover, some of the isolated intrabodies can reduce the neurotoxicity of mutant Htt in cell culture and animal models of HD. Thus, the production of antibodies and intrabodies has made a significant contribution to the understanding of HD pathogenesis and has introduced a novel strategy to treat this debilitating neurodegenerative disorder.

Item Type:Book Section
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Additional Information:© 2013 Springer Science+Business Media. Work cited from the authors’ laboratory was supported by the Hereditary Disease Foundation.
Funding AgencyGrant Number
Hereditary Disease FoundationUNSPECIFIED
Subject Keywords:mAb, scFv, Intrabodies, Huntingtin
Series Name:Methods in Molecular Biology
Issue or Number:1010
Record Number:CaltechAUTHORS:20130624-083018801
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Usage Policy:No commercial reproduction, distribution, display or performance rights in this work are provided.
ID Code:39040
Deposited By: Tony Diaz
Deposited On:24 Jun 2013 15:38
Last Modified:09 Nov 2021 23:42

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