Immunolocalization of prealbumin: Distribution in normal human tissue

Liddle, C N; Reid, W. A.; Kennedy, J. S.; Miller, Iain D.; Horne, C. H. W.

doi:10.1002/path.1711460205

Cited by 22 publications

(4 citation statements)

References 9 publications

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“…1985). TTR is synthesized mainly in the liver, choroid plexus and retinal pigment cells, and is secreted into plasma, cerebrospinal fluid, and vitreous body, respectively (Liddle et al . 1985; Herbert et al .…”

Section: Introductionmentioning

confidence: 99%

Inconsistency between hepatic expression and serum concentration of transthyretin in mice humanized at the transthyretin locus

Zhao

Araki

et al. 2008

Genes to Cells

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Human transthyretin (TTR) has about 110 variants, more than 90 of which are associated with human amyloidosis. Several groups have generated transgenic mice that carry various mutant TTR genes. However, formation of mouse/human TTR heterotetramers has been shown to be inhibitory to dissociation and subsequent amyloid formation. To avoid the effect of mouse Ttr and produce humanized mice carrying different TTR variants at high efficiency, we first produced a null allele in the mouse transthyretin locus using targeting vector that contained a neomycin resistance gene flanked by lox71 and loxP. Then, through Cre‐mediated recombination, we created a replacement allele that carried either a human normal (Val30) or mutant (Met30) TTR cDNA. This replacement resulted in a humanized TTR mouse with similar tissue‐specific profile of human TTR as that of the endogenous mouse Ttr gene. The expression levels of human TTR mRNA and protein in the liver of homozygous human TTR (Val30/Val30) mice were about twice those of heterozygous mouse/human TTR (+/Val30) mice. However, the serum human TTR levels in the Val30/Val30 mice were much less than those in the +/Val30 mice. This contradictory expression was due to unstable Val30 tetramers caused by low binding affinity to mouse retinol binding protein.

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Section: Introductionmentioning

confidence: 99%

Inconsistency between hepatic expression and serum concentration of transthyretin in mice humanized at the transthyretin locus

Zhao

Araki

et al. 2008

Genes to Cells

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show abstract

“…TTR is synthesized mainly in the liver [Smith and Goodman, 1971], but also in the epithelial cells in the choroid plexuses [Aleshire et al 1983;Dickson et al 1986] in the brain, in A-cells of the pancreatic islets [Jacobsson et al 1979;Jacobsson et al 1989;Liddle et al 1985] and retinal pigment epithelial cells [Ong et al 1994]. The concentration of TTR in plasma is about 0.3 g/l and in CSF 0.17 g/l.…”

Section: Ttrmentioning

confidence: 97%

Gene Therapy by Liver Transplantation and Single Stranded Oligonucleotides (SSOs) in Familial Amyloidotic Polyneuropathy (FAP)

Ando¹,

Nakamura

2006

CPG

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Liver transplantation has been considered as a promising therapy to halt the progression of clinical symptoms of progression in familial amyloidotic polyneuropathy (FAP) because most of transthyretin (TTR) is produced by the liver. In addition, domino liver transplantation using an FAP patient's liver has been performed because of shortage of domor livers. However, the use of liver transplantation as therapy for familial amyloidotic polyneuropathy (FAP) has given rise to several problems, an alternative treatment is needed. We have tried several other approaches: To suppress production of variant transthyretin (TTR) in the liver, we injected purified normal human TTR into patients with FAP. Production of variant TTR levels decreased significantly for 48 h. However, 1 week after the injection, production returned to levels that were almost the same as those before injection. TTR has a rapid turnover, so this method cannot be used to suppress production of the harmful protein for an extended time. An RNA/DNA chimera and single-stranded oligonucleotides (SSOs) were tested in vitro and in vivo in an attempt to repair the amyloidogenic TTR gene in the liver and retina. On the basis of results achieved so far, SSOs is a promising tool for gene therapy.

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“…TTR is synthesized mainly in the liver (35), but also in the epithelial cells in the choroid plexuses (36)(37) in the brain, in α-cells of the pancreatic islets (38)(39)(40) and retinal pigment epithelial cells (41)(42). The concentration of TTR in plasma is about 0.3 g/l and in CSF 0.17 g/l.…”

Section: Ttr: Structure Function and Genementioning

confidence: 99%

Novel methods for detecting amyloidogenic proteins in transthyretin related amyloidosis

Ando

Ueda²

2008

Front Biosci

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Transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP) is an autosomal dominant form of fatal hereditary amyloidosis. Until 25 years ago, tools for diagnosis of FAP were restricted to clinical manifestations and pathologic methods, and a small number of patients in the restricted endemic areas could be diagnosed with this disease. However, owing to progress in biochemical and molecular genetic analyses, this disease is now believed to occur worldwide. As of today, reports of about 100 different points of single or double mutations, or a deletion in the TTR gene have been published, and several different phenotypes of FAP have been documented, even for the same mutation in the TTR gene. Since liver transplantation has been established to halt the progression of FAP, rapid and reliable diagnostic system for FAP is needed. We present here a new diagnostic procedure for the disease using current methods of molecular genetics and protein chemistry.

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Immunolocalization of prealbumin: Distribution in normal human tissue

Cited by 22 publications

References 9 publications

Inconsistency between hepatic expression and serum concentration of transthyretin in mice humanized at the transthyretin locus

Inconsistency between hepatic expression and serum concentration of transthyretin in mice humanized at the transthyretin locus

Gene Therapy by Liver Transplantation and Single Stranded Oligonucleotides (SSOs) in Familial Amyloidotic Polyneuropathy (FAP)

Novel methods for detecting amyloidogenic proteins in transthyretin related amyloidosis

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