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Clinical Picture and Outcome of Transthyretin-Related Familial Amyloid Polyneuropathy (FAP) in Japanese Patients
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Shu-ichi Ikeda
Veröffentlicht/Copyright:
1. Juni 2005
Abstract
Familial amyloid polyneuropathy (FAP) was once considered to be a disease peculiar to endemic areas, but it is now recognized that FAP kindreds exist in worldwide places. The amyloid precursor of FAP is a variant form of transthyretin (TTR) with one amino acid substitution, which is ascribed to a mutation of TTR gene. Corresponding to the variety of the clinical phenotypes of FAP, more than 80 mutations have been identified as causative gene abnormality in this disease. Since the vast majority of TTR in serum is produced in the liver, liver transplantation has become widely accepted as a valuable treatment for FAP.
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Published Online: 2005-06-01
Published in Print: 2002-12-10
Copyright © 2002 by Walter de Gruyter GmbH & Co. KG
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Artikel in diesem Heft
- Author Index
- Subject Index
- Contents
- Historical Aspects and Perspectives in Transthyretin Research
- Transthyretin from Discovery to Now
- The Evolution of Transthyretin Synthesis in Vertebrate Liver, in Primitive Eukaryotes and in Bacteria
- The Evolution of Transthyretin Synthesis in the Choroid Plexus
- Hepatic Synthesis, Maturation and Complex Formation between Retinol-Binding Protein and Transthyretin
- Synthesis and Structural Analysis of the N-Terminal Domain of the Thyroid Hormone-Binding Protein Transthyretin
- Three-Dimensional Structure of the Transthyretin-Retinol-Binding Protein Complex
- Mechanisms of Molecular Recognition: Crystal Structure Analysis of Human and Rat Transthyretin Inhibitor Complexes
- Transthyretin in Fish: State of the Art
- The Effects of Endocrine-Disrupting Chemicals on Thyroid Hormone Binding to Xenopus laevis Transthyretin and Thyroid Hormone Receptor
- Clinical Picture and Outcome of Transthyretin-Related Familial Amyloid Polyneuropathy (FAP) in Japanese Patients
- Laboratory Assessment of Transthyretin Amyloidosis
- Amyloid: Morphology and Toxicity
- Historical Overview of Analytical Methods for the Measurement of Transthyretin
- Nutritional Regulation of Visceral Markers in Rat Liver and Cultured Hepatoma Cells
- Significance of Transthyretin in Protein Metabolism
- Transthyretin as a Thyroid Hormone Carrier: Function Revisited
- The Molar Ratio of Retinol-Binding Protein to Transthyretin in the Assessment of Vitamin A Status in Adults. Proposal of a Cut-off Point
- Observations with Regard to the National Kidney Foundation K/DOQI Clinical Practice Guidelines Concerning Serum Transthyretin in Chronic Renal Failure
- Metabolism and Clinical Interest of Serum Transthyretin (Prealbumin) in Dialysis Patients
- Protein Status in Pancreatitis – Transthyretin Is a Sensitive Biomarker of Malnutrition in Acute and Chronic Pancreatitis
- Assessment of Nutritional Status in Organ Transplant: Is Transthyretin a Reliable Indicator?
- Body Composition and Nutritional Parameters in HIV and AIDS Patients
- C-Reactive Protein to Transthyretin Ratio for the Early Diagnosis and Follow-up of Postoperative Infection
- The Prognostic Value of Nutritional and Inflammatory Indices in Critically Ill Patients with Acute Respiratory Failure
- Transthyretin: Its Response to Malnutrition and Stress Injury. Clinical Usefulness and Economic Implications
- Transthyretin Measurement as a Screening Tool for Protein Calorie Malnutrition in Emergency Hospital Admissions
- Outcomes of Continuous Process Improvement of a Nutritional Care Program Incorporating TTR Measurement
- The Role of Visceral Protein Markers in Protein Calorie Malnutrition
- Acknowledgement
- Meetings
Artikel in diesem Heft
- Author Index
- Subject Index
- Contents
- Historical Aspects and Perspectives in Transthyretin Research
- Transthyretin from Discovery to Now
- The Evolution of Transthyretin Synthesis in Vertebrate Liver, in Primitive Eukaryotes and in Bacteria
- The Evolution of Transthyretin Synthesis in the Choroid Plexus
- Hepatic Synthesis, Maturation and Complex Formation between Retinol-Binding Protein and Transthyretin
- Synthesis and Structural Analysis of the N-Terminal Domain of the Thyroid Hormone-Binding Protein Transthyretin
- Three-Dimensional Structure of the Transthyretin-Retinol-Binding Protein Complex
- Mechanisms of Molecular Recognition: Crystal Structure Analysis of Human and Rat Transthyretin Inhibitor Complexes
- Transthyretin in Fish: State of the Art
- The Effects of Endocrine-Disrupting Chemicals on Thyroid Hormone Binding to Xenopus laevis Transthyretin and Thyroid Hormone Receptor
- Clinical Picture and Outcome of Transthyretin-Related Familial Amyloid Polyneuropathy (FAP) in Japanese Patients
- Laboratory Assessment of Transthyretin Amyloidosis
- Amyloid: Morphology and Toxicity
- Historical Overview of Analytical Methods for the Measurement of Transthyretin
- Nutritional Regulation of Visceral Markers in Rat Liver and Cultured Hepatoma Cells
- Significance of Transthyretin in Protein Metabolism
- Transthyretin as a Thyroid Hormone Carrier: Function Revisited
- The Molar Ratio of Retinol-Binding Protein to Transthyretin in the Assessment of Vitamin A Status in Adults. Proposal of a Cut-off Point
- Observations with Regard to the National Kidney Foundation K/DOQI Clinical Practice Guidelines Concerning Serum Transthyretin in Chronic Renal Failure
- Metabolism and Clinical Interest of Serum Transthyretin (Prealbumin) in Dialysis Patients
- Protein Status in Pancreatitis – Transthyretin Is a Sensitive Biomarker of Malnutrition in Acute and Chronic Pancreatitis
- Assessment of Nutritional Status in Organ Transplant: Is Transthyretin a Reliable Indicator?
- Body Composition and Nutritional Parameters in HIV and AIDS Patients
- C-Reactive Protein to Transthyretin Ratio for the Early Diagnosis and Follow-up of Postoperative Infection
- The Prognostic Value of Nutritional and Inflammatory Indices in Critically Ill Patients with Acute Respiratory Failure
- Transthyretin: Its Response to Malnutrition and Stress Injury. Clinical Usefulness and Economic Implications
- Transthyretin Measurement as a Screening Tool for Protein Calorie Malnutrition in Emergency Hospital Admissions
- Outcomes of Continuous Process Improvement of a Nutritional Care Program Incorporating TTR Measurement
- The Role of Visceral Protein Markers in Protein Calorie Malnutrition
- Acknowledgement
- Meetings
