Please use this identifier to cite or link to this item: http://hdl.handle.net/10125/33668

Misincorporation of Beta-Methylamino-L-Alanine Into Protein: A Potential Mechanism for Motor Neuron Disease

File SizeFormat 
Liu_Jenny_Senior_Honors_Thesis.pdf735.69 kBAdobe PDFView/Open

Item Summary

Title: Misincorporation of Beta-Methylamino-L-Alanine Into Protein: A Potential Mechanism for Motor Neuron Disease
Authors: Liu, Jenny
Advisor: Hemscheidt, Thomas
Keywords: beta-methylamino-L-alanine (BMAA)
misincorporation
Issue Date: 26 Sep 2014
Publisher: University of Hawaii at Manoa
Abstract: Imagine living in an immobilized body, while being fully aware of your surroundings. This is the experience of many motor neuron disease afflicted patients, especially those with amyotrophic lateral sclerosis (ALS). ALS-Parkinsonism Dementia complex (ALS-PDC) is a form of ALS with an abnormally high incidence in members of certain ethnicities. An ethnobotanical connection to nutrition involving the cyanobacterial neurotoxin beta- methylamino-L-alanine (BMAA) has long been postulated. Specifically, incorporation of BMAA into human protein has been suggested to contribute significantly to the etiology of ALS-PDC. Using an in-vivo approach, this research project focused on determining whether BMAA is transformed into high molecular weight substances such as proteins. The uptake and metabolic fate of BMAA was followed within a prokaryotic model system, a cyanobacterium, during the logarithmic growth phase. Prokaryotes generally have higher error rates for the misincorporation of non-proteinogenic amino acids into protein than eukaryotes. Observation of BMAA in the protein fraction would therefore provide a first estimate of the potential significance of the misincorporation of BMAA in a eukaryotic system. Chemical analysis showed that about 1% of the initial amount of BMAA added to the cell culture was present within intracellular, hydrolyzable, low molecular weight compounds below 10 kilodaltons, and only 6 × 10-4 %, if any, was present in the high molecular mass, i.e. protein, form. This suggests that intracellular protein is not a primary reservoir of BMAA in this model system and, by extension, in eukaryotes, and that an unexpectedly large percentage of BMAA, 98%, is metabolized. Imagine living in an immobilized body, while being fully aware of your surroundings.
Pages/Duration: v, 35 pages
URI/DOI: http://hdl.handle.net/10125/33668
Rights: All UHM Honors Projects are protected by copyright. They may be viewed from this source for any purpose, but reproduction or distribution in any format is prohibited without written permission from the copyright owner.
Appears in Collections:Honors Projects for Chemistry



Items in ScholarSpace are protected by copyright, with all rights reserved, unless otherwise indicated.