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Mutant protein in Huntington disease is resistant to proteolysis in affected brain
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Journal Article
Mutant protein in Huntington disease is resistant to proteolysis in affected brain
2001
Overview
The cause of Huntington disease pathophysiology is unknown, but a major hypothesis suggests that toxicity arises from the cleavage and accumulation of amino-terminal fragments containing an expanded polyglutamine region. In evaluating huntingtin protein (HD) from human brain, transgenic animals and cells, we observed, unexpectedly, that mutant HD is more resistant to proteolysis than normal HD. The N-terminal cleavage fragments we observed arise from the processing of normal HD and are sequestered by full-length mutant HD. Our results support a model in which inhibition of proteolysis of mutant HD leads to aggregation and toxicity through the sequestering of important targets, including normal HD.
Publisher
Nature Publishing Group US,Nature Publishing Group
Subject
/ Animal Genetics and Genomics
/ Animals
/ Biological and medical sciences
/ Biomedical and Life Sciences
/ Brain
/ Complications and side effects
/ Female
/ Fundamental and applied biological sciences. Psychology
/ Humans
/ Huntington Disease - genetics
/ Huntington Disease - metabolism
/ Huntington Disease - pathology
/ Huntington Disease - physiopathology
/ Male
/ Mice
/ Molecular and cellular biology
/ Mutants
/ Nerve Tissue Proteins - chemistry
/ Nerve Tissue Proteins - genetics
/ Nerve Tissue Proteins - metabolism
/ Nerve Tissue Proteins - toxicity
/ Nuclear Proteins - chemistry
/ Nuclear Proteins - metabolism
/ Peptide Fragments - chemistry
/ Peptide Fragments - metabolism
/ Peptides
/ Protein Structure, Quaternary
/ Proteins
/ Toxicity
