{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,4,22]],"date-time":"2026-04-22T08:26:36Z","timestamp":1776846396770,"version":"3.51.2"},"reference-count":82,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"8","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2012,8]]},"abstract":"<jats:sec>\n            <jats:title>Summary<\/jats:title>\n            <jats:p>The amyloidoses are protein-misfolding disorders associated with progressive organ dysfunction. Immunoglobulin light chain is the most common, amyloid A the longest recognized, and transthyretin-associated amyloidosis (ATTR) the most frequent inherited systemic form. Although ATTR, an autosomal-dominant disease, is associated with at least 100 different transthyretin (TTR) mutations, the single amino-acid substitution of methionine for valine at position 30 is the most common mutation. Each variant has a different organ involvement, although clinical differences attributed to environmental and genetic factors exist within the same mutation. Peripheral neuropathy and cardiomyopathy are broadly described, and insights into disease reveal that kidney impairment and proteinuria are also clinical features. This review combines clinical and laboratory findings of renal involvement from the main geographic regions of disease occurrence and for different mutations of TTR. Fifteen nephropathic variants have been described, but the TTR V30M mutation is the best documented. Nephropathy affects patients with late-onset neuropathy, low penetrance in the family, and cardiac dysrhythmias. Microalbuminuria can be the disorder\u2019s first presentation, even before the onset of neuropathy. Amyloid renal deposits commonly occur, even in the absence of urinary abnormalities. The experience with renal replacement therapy is based on hemodialysis, which is associated with poor survival. Because TTR is synthesized mainly in the liver, liver transplantation has been considered an acceptable treatment; simultaneous liver-kidney transplantation is recommended to avoid recurrence of nephropathy. In addition, the kidney-safety profile of new drugs in development may soon be available.<\/jats:p>\n          <\/jats:sec>","DOI":"10.2215\/cjn.08720811","type":"journal-article","created":{"date-parts":[[2012,4,28]],"date-time":"2012-04-28T00:14:07Z","timestamp":1335572047000},"page":"1337-1346","source":"Crossref","is-referenced-by-count":130,"title":["Transthyretin Amyloidosis and the Kidney"],"prefix":"10.2215","volume":"7","author":[{"given":"Lu\u00edsa","family":"Lobato","sequence":"first","affiliation":[]},{"given":"Ana","family":"Rocha","sequence":"additional","affiliation":[]}],"member":"276","reference":[{"key":"R1-20-20230811","doi-asserted-by":"crossref","first-page":"408","DOI":"10.1093\/brain\/75.3.408","article-title":"A peculiar form of peripheral neuropathy; familiar atypical generalized amyloidosis with special involvement of the peripheral nerves.","volume":"75","author":"Andrade","year":"1952","journal-title":"Brain"},{"key":"R2-20-20230811","doi-asserted-by":"crossref","first-page":"6796","DOI":"10.1016\/S0021-9258(19)42128-5","article-title":"The amino acid sequence of human plasma prealbumin.","volume":"249","author":"Kanda","year":"1974","journal-title":"J Biol Chem"},{"key":"R3-20-20230811","first-page":"4697","article-title":"Transcriptional control of the mouse prealbumin (transthyretin) gene: Both promoter sequences and a distinct enhancer are cell specific.","volume":"6","author":"Costa","year":"1986","journal-title":"Mol Cell Biol"},{"key":"R4-20-20230811","doi-asserted-by":"crossref","first-page":"1215","DOI":"10.1093\/oxfordjournals.jbchem.a121826","article-title":"Analyses of prealbumin mRNAs in individuals with familial amyloidotic polyneuropathy.","volume":"100","author":"Mita","year":"1986","journal-title":"J Biochem"},{"key":"R5-20-20230811","first-page":"313","article-title":"Retinol-binding protein, prealbumin, and vitamin A transport.","volume":"5","author":"Goodman","year":"1976","journal-title":"Prog Clin Biol Res"},{"key":"R6-20-20230811","doi-asserted-by":"crossref","first-page":"753","DOI":"10.1016\/0006-291X(85)91956-4","article-title":"Localization of the human prealbumin gene to chromosome 18.","volume":"129","author":"Wallace","year":"1985","journal-title":"Biochem Biophys Res Commun"},{"key":"R7-20-20230811","doi-asserted-by":"crossref","first-page":"385","DOI":"10.1038\/labinvest.3780246","article-title":"Amyloid and nonfibrillar deposits in mice transgenic for wild-type human transthyretin: A possible model for senile systemic amyloidosis.","volume":"81","author":"Teng","year":"2001","journal-title":"Lab Invest"},{"key":"R8-20-20230811","doi-asserted-by":"crossref","first-page":"27","DOI":"10.1111\/j.1399-0004.1992.tb03131.x","article-title":"Amyloidogenic and non-amyloidogenic transthyretin Asn 90 variants.","volume":"42","author":"Alves","year":"1992","journal-title":"Clin Genet"},{"key":"R9-20-20230811","doi-asserted-by":"crossref","first-page":"242","DOI":"10.1111\/j.1399-0004.1991.tb03085.x","article-title":"Biochemical effect of liver transplantation in two Swedish patients with familial amyloidotic polyneuropathy (FAP-met30).","volume":"40","author":"Holmgren","year":"1991","journal-title":"Clin Genet"},{"key":"R10-20-20230811","first-page":"1","article-title":"Familial amyloidosis with polyneuropathy. 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