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Progression of transthyretin amyloid neuropathy after liver transplantation

Progression of transthyretin amyloid neuropathy after liver transplantation Progression of transthyretin amyloid neuropathy after liver transplantation Juris J. Liepnieks, PhD ABSTRACT Lucy Q. Zhang, BS Objective: To biochemically characterize peripheral nerve amyloid in subjects with transthyretin Merrill D. Benson, MD (TTR) amyloidosis and assess effect of orthotopic liver transplantation (OLT) on progression of neuropathy. Methods: Amyloid fibrils were isolated from peripheral nerve tissues of 6 patients with TTR amy- Address correspondence and loidosis who were heterozygous for an amyloid-associated TTR mutation. Ratio of variant to wild- reprint requests to Dr. Merrill D. Benson, Indiana University type TTR in the fibrils was determined by amino acid sequencing of tryptic peptides containing School of Medicine, Department either the variant amino acid residue or the corresponding normal amino acid. of Pathology and Laboratory Medicine, 635 Barnhill Drive, Results: Amyloid fibrils from 3 subjects who died without having received a liver transplant were MS-128, Indianapolis, IN 46202- composed of 60%– 65% variant TTR and 35%– 40% wild-type. Amyloid fibrils from a subject [email protected] who died 5 years after liver transplantation contained 25% variant and 75% wild-type TTR. Conclusion: Ratios of variant to wild-type TTR in amyloid patients heterozygous for an amyloid- associated TTR mutation are similar to published ratios for amyloid http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png Neurology Wolters Kluwer Health

Progression of transthyretin amyloid neuropathy after liver transplantation

Benson, Merrill D.
Neurology , Volume 75 (4) – Jul 1, 2010
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References (17)

ISSN
0028-3878
eISSN
1526-632X
DOI
10.1212/WNL.0b013e3181ea15d4
pmid
20660862
Publisher site
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Abstract

Progression of transthyretin amyloid neuropathy after liver transplantation Juris J. Liepnieks, PhD ABSTRACT Lucy Q. Zhang, BS Objective: To biochemically characterize peripheral nerve amyloid in subjects with transthyretin Merrill D. Benson, MD (TTR) amyloidosis and assess effect of orthotopic liver transplantation (OLT) on progression of neuropathy. Methods: Amyloid fibrils were isolated from peripheral nerve tissues of 6 patients with TTR amy- Address correspondence and loidosis who were heterozygous for an amyloid-associated TTR mutation. Ratio of variant to wild- reprint requests to Dr. Merrill D. Benson, Indiana University type TTR in the fibrils was determined by amino acid sequencing of tryptic peptides containing School of Medicine, Department either the variant amino acid residue or the corresponding normal amino acid. of Pathology and Laboratory Medicine, 635 Barnhill Drive, Results: Amyloid fibrils from 3 subjects who died without having received a liver transplant were MS-128, Indianapolis, IN 46202- composed of 60%– 65% variant TTR and 35%– 40% wild-type. Amyloid fibrils from a subject [email protected] who died 5 years after liver transplantation contained 25% variant and 75% wild-type TTR. Conclusion: Ratios of variant to wild-type TTR in amyloid patients heterozygous for an amyloid- associated TTR mutation are similar to published ratios for amyloid

Journal

Neurology – Wolters Kluwer Health

Published: Jul 1, 2010

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