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| transthyretinSummaryGene Symbol: transthyretin Description: transthyretin Alias: CTS, CTS1, HsT2651, PALB, TBPA, ATTR, carpal tunnel syndrome 1, prealbumin, amyloidosis type I, thyroxine-binding prealbumin Species: human Top Publications
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The Endoplasmic Reticulum-associated Degradation of Transthyretin Variants Is Negatively Regulated by BiP in Mammalian CellsSeiko Susuki
Department of Molecular Medicine, Faculty of Medical and Pharmaceutical Sciences, Global Centers of Excellence Cell Fate Regulation Research and Education Unit, Kumamoto University, 5 1 Oe honmachi, Kumamoto 862 0973, Japan
J Biol Chem 284:8312-21. 2009Amyloid fibril formation of mutant transthyretin (TTR) that causes familial amyloid polyneuropathy occurs in the extracellular space. Thus, secretion of TTR variants contributes to the pathogenesis of amyloidosis...
Oxidation inhibits amyloid fibril formation of transthyretinSimin D Maleknia
School of Biological, Earth and Environmental Sciences, University of New South Wales, Sydney, NSW, Australia
FEBS J 273:5400-6. 2006..b>Transthyretin (TTR) is a soluble human plasma protein...
Tissue damage in the amyloidoses: Transthyretin monomers and nonnative oligomers are the major cytotoxic species in tissue cultureNatalia Reixach
Division of Rheumatology Research, Department of Molecular and Experimental Medicine, The Scripps Research Institute, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
Proc Natl Acad Sci U S A 101:2817-22. 2004The transthyretin (TTR) amyloidoses are human diseases in which the misfolded TTR protein aggregates in tissues with subsequent visceral, peripheral, and autonomic nerve dysfunction...
Amyloid fibril composition and transthyretin gene structure in senile systemic amyloidosisA Gustavsson
Department of Pathology I, Linkoping University, Sweden
Lab Invest 73:703-8. 1995In many different forms of amyloidosis, transthyretin (TTR) comprises the major amyloid fibril protein. In the familial forms, various TTR mutations are linked to disease...
Serum transthyretin levels in Swedish TTR V30M carriersJoel Buxbaum
Department of Molecular and Experimental Medicine, WM Keck Autoimmune Disease Center, The Scripps Research Institute, University of Umea, Umea, Sweden
Amyloid 17:83-5. 2010Serum transthyretin (TTR) levels have been reported to be reduced in Portuguese and Japanese patients with TTR V30M familial amyloidotic polyneuropathy and pre-symptomatic carriers of the allele as well as in the carriers of a number of ..
Dimeric transthyretin variant assembles into spherical neurotoxinsKimiaki Matsubara
Faculty of Pharmaceutical Sciences, Toyama Medical and Pharmaceutical University, Toyama 930 0194, Japan
Biochemistry 44:3280-8. 2005Familial amyloidotic polyneuropathy is a hereditary autosomal-dominant disease in which the deposited transthyretin fibrils are derived from amyloidogenic mutation...
Transthyretin mutations in health and diseaseM J Saraiva
Instituto de Ciencias Biomedicas, Universidade do Porto, Portugal
Hum Mutat 5:191-6. 1995To date, over 40 different mutations in transthyretin (TTR) have been associated with amyloid deposition. The major unresolved problem is the correlation between the clinical heterogeneity and the genetic heterogeneity...
On the origin of the transthyretin Val30Met familial amyloid polyneuropathyC Zaros
Department of Neurology, Hopital Henri Mondor, 94000 Creteil, France
Ann Hum Genet 72:478-84. 2008b>Transthyretin (TTR) familial amyloid polyneuropathy is a severe autosomal dominant neuropathy of adulthood, frequently linked to the pathogenic Val30Met variant of the TTR gene...
Structure of a complex of two plasma proteins: transthyretin and retinol-binding proteinH L Monaco
Department of Genetics, University of Pavia, Italy
Science 268:1039-41. 1995The three-dimensional structure of the complex formed by two plasma proteins, transthyretin and retinol-binding protein, was determined from x-ray diffraction data to a nominal resolution of 3.1 angstroms...
Heterogeneity of penetrance in familial amyloid polyneuropathy, ATTR Val30Met, in the Swedish populationUrban Hellman
Medical and Clinical Genetics, Umea University, Umea, Sweden
Amyloid 15:181-6. 2008b>Transthyretin (TTR) familial amyloid polyneuropathies (FAP) are autosomal dominant devastating afflictions. They were first described in Portugal, later in Japan and Sweden and are now recognized worldwide...
Haplotypes and DNA sequence variation within and surrounding the transthyretin gene: genotype-phenotype correlations in familial amyloid polyneuropathy (V30M) in Portugal and SwedenMiguel Luz Soares
Division of Rheumatology Research and the W M Keck Autoimmune Disease Center, Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA, USA
Eur J Hum Genet 12:225-37. 2004..polyneuropathy (FAP) is a lethal autosomal dominant disorder in which fibrils derived from mutant forms of transthyretin (TTR), the normal plasma carrier of thyroxine (T(4)) and retinol-binding protein, are deposited in tissues...
Structure of prealbumin: secondary, tertiary and quaternary interactions determined by Fourier refinement at 1.8 AC C Blake
J Mol Biol 121:339-56. 1978
An aberrant protein complex in CSF as a biomarker of Alzheimer diseaseM A Lovell
Department of Chemistry, Sanders Brown Center on Aging and Alzheimer s Disease Center, University of Kentucky, Lexington, KY 40536 0230, USA
Neurology 70:2212-8. 2008To determine if an aberrant protein complex consisting of prostaglandin-d-synthase (PDS) and transthyretin (TTR) in CSF differentiates between subjects with Alzheimer disease (AD) and normal control (NC) subjects.
The x-ray crystal structure refinements of normal human transthyretin and the amyloidogenic Val-30-->Met variant to 1.7-A resolutionJ A Hamilton
Department of Biochemistry and Molecular Biology, Indiana University School of Medicine, Indianapolis 46202 5122
J Biol Chem 268:2416-24. 1993The x-ray crystal structures of normal human transthyretin (prealbumin) and the amyloidogenic Val-30-Met variant have been refined at 1.7-A resolution to R-values of 0.168 and 0...
Impact of homozygosity for an amyloidogenic transthyretin mutation on phenotype and long term outcomeG Holmgren
J Med Genet 42:953-6. 2005Although amyloidogenic transthyretin (ATTR) mutations are common in several populations, such as black Americans, the small number of diagnosed patients homozygous for TTR amyloid and the short follow up in most studies has until now ..
Susceptibility and modifier genes in Portuguese transthyretin V30M amyloid polyneuropathy: complexity in a single-gene diseaseMiguel L Soares
Department of Molecular and Experimental Medicine, Division of Rheumatology Research and the WM Keck Autoimmune Disease Center, The Scripps Research Institute, La Jolla, CA 92037, USA
Hum Mol Genet 14:543-53. 2005Familial amyloid polyneuropathy type I is an autosomal dominant disorder caused by mutations in the transthyretin (TTR) gene; however, carriers of the same mutation exhibit variability in penetrance and clinical expression...
DJ-1 degrades transthyretin and an inactive form of DJ-1 is secreted in familial amyloidotic polyneuropathyShizuyo Koide-Yoshida
Graduate School of Pharmaceutical Sciences, Hokkaido University, Sapporo 060 8589, Japan
Int J Mol Med 19:885-93. 2007..DJ-1 has a protease-like structure and transthyretin (TTR), a protein causing familial amyloidotic polyneuropathy (FAP), was identified as a substrate for DJ-1 ..
[Immune, inflammatory, and nutritional protein profile in children with iron deficiency in Côte d'Ivoire]Houphouët Félix Yapi
Laboratoire de pharmacodynamie biochimique, UFR Biosciences, Université de Cocody Abidjan, Abidjan 22, Cote d Ivoire
Sante 19:25-8. 2009..05. Iron deficiency was also associated with reduced levels of thyroxine-binding prealbumin (TBPA) and albumin (p < 0.05). Inflammatory proteins did not differ significantly between the two groups (p > 0.05)...
[The importance of TTR met 30 in the diagnosis of familial amyloidotic polyneuropathy in the absence of a positive family history]M Santos
Unidade de Neurologia e Neurofisiologia, , Portugal
Rev Neurol 30:929-31. 2000..polyneuropathy type I is an hereditary autosomal dominant disease, characterized by having a mutant transthyretin (TTR met 30)...
Recombinant transthyretin purification and competitive binding with organohalogen compounds in two gull species (Larus argentatus and Larus hyperboreus)Francisco Ucán-Marin
National Wildlife Research Centre, Carleton University, Ottawa, Ontario, K1A 0H3, Canada
Toxicol Sci 107:440-50. 2009..Such compounds can potentially perturb hormone transport via binding interactions with proteins such as transthyretin (TTR, prealbumin)...
Inhibition of serum protein binding of thyroxine in a hypothyroid patient with familial dysalbuminemic hyperthyroxinemiaB N Premachandra
Research Service, Veterans Administration Medical Center, St Louis, MO 63125, USA
Clin Biochem 29:85-8. 1996..To investigate unusual free thyroxine (FT4) responses to T4 replacement doses in a hypothyroid patient with familial dysalbuminemic hyperthyroxinemia (FDH)...
Assessing the role of ortho-substitution on polychlorinated biphenyl binding to transthyretin, a thyroxine transport proteinK R Chauhan
Curriculum in Toxicology, University of North Carolina, Chapel Hill, North Carolina, USA
Toxicol Appl Pharmacol 162:10-21. 2000..Previous structure-activity relationship (SAR) studies on second messengers and transthyretin (TTR; prealbumin) binding focused little attention on the ortho-substituted PCBs...
Impact of varying levels of protein intake on protein status indicators after gastric bypass in patients with multiple complications requiring nutritional supportElizabeth Rinaldi Schinkel
Department of Medical Nutrition Therapy, Poudre Valley Hospital, Fort Collins, CO 80524, and Department of Public Health and Preventive Medicine, Oregon Health and Science University, Portland, OR, USA
Obes Surg 16:24-30. 2006..This study assesses the effect of varying protein-calorie intake in complicated patients after RYGBP on two markers of protein status: thyroxine-binding prealbumin (TBPA) and serum albumin.
Evolution of the thyroid hormone-binding protein, transthyretinD M Power
Centro de Ciências de MAR CCMAR, Universidade do Algarve, Campus de Gambelas, 8000 Faro, Portugal
Gen Comp Endocrinol 119:241-55. 2000b>Transthyretin (TTR) belongs to a group of proteins, which includes thyroxine-binding globulin and albumin, that bind to and transport thyroid hormones in the blood...
Transthyretin from discovery to nowJacob Robbins
Genetics and Biochemistry Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, Maryland 20892, USA
Clin Chem Lab Med 40:1183-90. 2002As introduction to the First International Congress on Transthyretin in Health and Disease, this lecture traces the origin of the subjectfrom the discovery in the 1950s that a serum protein migrating ahead of albumin in an electrical ..
Binding activities of thyroxine binding globulin versus thyroxine binding prealbumin in rat sera: differential modulation by thyroid hormone ligands, oleic acid and pharmacological drugsL Savu
U 224, INSERM, Faculte de Medecine Xavier Bichat, Paris, France
Biochem Biophys Res Commun 159:919-26. 1989..and electrophoretic techniques to compare the binding properties of thyroxine binding globulin and thyroxine binding prealbumin in rat sera...
Observations with regard to the National Kidney Foundation K/DOQI clinical practice guidelines concerning serum transthyretin in chronic renal failureJoel D Kopple
Division of Nephrology and Hypertension and the Research and Education Institute at Harbor UCLA Medical Center, Torrance, CA 90502, USA
Clin Chem Lab Med 40:1308-12. 2002..quot; Prealbumin, also known as serum transthyretin (TTR), was not recommended as a nutritional parameter of the same usefulness as the serum albumin...
Cloning of putative piscine (Sparus aurata) transthyretin: developmental expression and tissue distributionB Funkenstein
Israel Oceanographic and Limnological Research, National Institute of Oceanography, Tel Shikmona, Haifa, Israel
Mol Cell Endocrinol 157:67-73. 1999cDNA encoding putative transthyretin (prealbumin, TTR) was cloned from liver of the marine fish Sparus aurata. The cDNA contains an open reading frame of 453 nt, encoding for a TTR precursor of 151 amino acids...
Electrically neutral microheterogeneity of human plasma transthyretin (prealbumin) detected by isoelectric focusing in urea gradientsK Altland
Institut fur Humangenetik, Justus Liebig Universitat, Giessen, Germany
Electrophoresis 20:1349-64. 1999Mutants of the human plasma transthyretin (TTR, prealbumin) have attracted interest due to their rather frequent association with the autosomal dominant disease familial amyloidotic polyneuropathy (FAP)...
[Fibril-forming proteins: the amyloidosis. New hopes for a disease that cardiologists must know]Eloisa Arbustini
Laboratorio di Diagnostica Molecolare, Area Trapiantologica di Ricerca, IRCCS Policlinico San Matteo, Dipartimento di Biochimica, Universita degli Studi, Pavia
Ital Heart J Suppl 3:590-7. 2002..In transthyretin (TTR) amyloidosis, the non-senile forms are autosomal dominant diseases caused by defective proteins ..
Cutaneous lymphatic amyloid deposits in "Hungarian-type" familial transthyretin amyloidosis: a case reportTibor Harkany
Department of Molecular Neurobiology, University of Groningen, PO Box 14, Kerklaan 30, NL 9750 AA Haren, The Netherlands
Br J Dermatol 146:674-9. 2002Multiple transthyretin (TTR) mutations have recently been identified and implicated in the development of familial systemic amyloidoses, but early diagnosis of these disorders is still largely unresolved...
Chain reaction of amyloid fibril formation with induction of basement membrane in familial amyloidotic polyneuropathyYohei Misumi
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto 860 081, Japan
J Pathol 219:481-90. 2009..FAP) is characterized by extracellular deposition of amyloid fibrils caused by a point mutation in the transthyretin (TTR) gene...
Matrix metalloproteinases and their tissue inhibitors in cardiac amyloidosis: relationship to structural, functional myocardial changes and to light chain amyloid depositionAndreia Biolo
Whitaker Cardiovascular Institute, Boston University School of Medicine, 715 Albany Street, Boston, MA 02118, USA
Circ Heart Fail 1:249-57. 2008..prognosis compared with non-light chain cardiac amyloidoses (ie, forms associated with wild-type or mutated transthyretin [TTR])...
Risk of transmission of systemic transthyretin amyloidosis after domino liver transplantationLaura Llado
Departments of Surgery, Liver Transplant Unit, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain
Liver Transpl 16:1386-92. 2010Recent reports of the transmission of systemic transthyretin (TTR) amyloidosis after domino liver transplantation (DLT) using grafts from patients with familial amyloid polyneuropathy (FAP) have raised concerns about the procedure...
Risk of transmission of systemic transthyretin amyloidosis after domino liver transplantationLaura Llado
Departments of Surgery, Liver Transplant Unit, Hospital Universitari de Bellvitge, IDIBELL, Barcelona, Spain
Liver Transpl 16:1386-92. 2010Recent reports of the transmission of systemic transthyretin (TTR) amyloidosis after domino liver transplantation (DLT) using grafts from patients with familial amyloid polyneuropathy (FAP) have raised concerns about the procedure...
Pathogenesis of and therapeutic strategies to ameliorate the transthyretin amyloidosesYoshiki Sekijima
Department of Neurology, Shinshu University School of Medicine, 3 1 1 Asahi, Matsumoto 390 8621, Japan
Curr Pharm Des 14:3219-30. 2008b>Transthyretin (TTR) is a homotetrameric serum and cerebrospinal fluid protein that transports both thyroxine (T(4)) and the retinol-retinol binding protein complex (holoRBP)...
Amyloid and nonfibrillar deposits in mice transgenic for wild-type human transthyretin: a possible model for senile systemic amyloidosisM H Teng
Research Service, New York Department of Veterans Affairs Medical Center, New York, USA
Lab Invest 81:385-96. 2001The human serum protein transthyretin (TTR) is highly fibrillogenic in vitro and is the fibril precursor in both autosomal dominant (familial amyloidotic polyneuropathy [FAP] and familial amyloidotic cardiomyopathy [FAC]) and sporadic (..
Liver transplantation for hereditary transthyretin amyloidosisO B Suhr
Department of Medicine, Umea University Hospital, Umea, Sweden
Liver Transpl 6:263-76. 2000b>Transthyretin (TTR) amyloidosis is the most common form of hereditary amyloidosis...
Familial ATTR amyloidosis: microalbuminuria as a predictor of symptomatic disease and clinical nephropathyLuísa Lobato
Department of Nephrology and Centro de Estudos de Paramiloidose, Hospital Geral de Santo António and Institute for Molecular and Cell Biology, Porto, Portugal
Nephrol Dial Transplant 18:532-8. 2003Portuguese type familial amyloid polyneuropathy (FAP) is a neuropathic amyloidosis caused by a mutant transthyretin (TTR). Varying degrees of renal involvement have been reported...
Sequence, genetic analysis, and expression of Actinobacillus pleuropneumoniae transferrin receptor genesG C Gonzalez
Department of Microbiology and Infectious Diseases, University of Calgary, AB, Canada
Microbiology 141:2405-16. 1995The tbpA and tbpB genes encoding the transferrin receptor proteins Tbp1 and Tbp2 from a serotype 7 strain of Actinobacillus pleuropneumoniae were cloned, sequenced, and expressed in Escherichia coli...
A dynamic model of the meningococcal transferrin receptorI C Boulton
Centre for Applied Microbiology and Research, Salisbury, SP4 0JG, UK
J Theor Biol 198:497-505. 1999..It has been established that transferrin-binding proteins (designated TbpA and TbpB) form a discrete, specific complex which may be composed of a transmembrane species (composed of the TbpA ..
Transferrin-binding protein B isolated from Neisseria meningitidis discriminates between apo and diferric human transferrinI C Boulton
Metalloprotein Research Group, Division of Biochemistry and Molecular Biology, United Medical and Dental School of Guy s and St Thomas s Hospitals, Guy s Hospital, London SE1 9RT
Biochem J 334:269-73. 1998..To characterize this process, functionally active meningococcal transferrin-binding protein A (TbpA) and TbpB have been purified from N. meningitidis using a novel purification procedure...
Thyroid function in sojourners and acclimatised low landers at high altitude in manR C Sawhney
Defence Institute of Physiology and Allied Sciences, Delhi Cantonment, India
Horm Metab Res 23:81-4. 1991..01) at HA, whereas plasma rT3, TBG and T4 binding capacities of TBG and TBPA did not show any appreciable change...
Neisseria meningitidis expressing transferrin binding proteins of Actinobacillus pleuropneumoniae can utilize porcine transferrin for growthD J Litt
Institute of Infections and Immunity, Queen s Medical Centre, Nottingham, NG7 2UH, United Kingdom
Infect Immun 68:550-7. 2000..or porcine transferrin because of loss of both transferrin binding proteins (Tbp) A and B [strain B16B6(Str(r))/tbpA(-)B(-)] and (ii) an ability to bind porcine transferrin but not human transferrin [strain B16B6(Str(r))/tbpA(ap)B(..
Low serum levels of prohepcidin, but not hepcidin-25, are related to anemia in familial amyloidosis TTR V30MIdalina Beirão
Department of Nephrology, HGSA, Santo Antonio General Hospital, Porto, Portugal
Blood Cells Mol Dis 41:175-8. 2008..In general, the decreased observed/expected EPO levels in FAP-I correlated with the prohepcidin levels, therefore raising the possibility that a common defect in these two hormones may be somehow involved in the genesis of the disease...
Evidence for a common gene pool and frequent recombinational exchange of the tbpBA operon in Mannheimia haemolytica, Mannheimia glucosida and Bibersteinia trehalosiInkyoung Lee
Institute of Infection, Immunity and Inflammation, College of Medical, Veterinary and Life Sciences, Sir Graeme Davies Building, 120 University Place, University of Glasgow, Glasgow G12 8TA, UK
Microbiology 157:123-35. 2011..A total of 27 tbpB and 20 tbpA alleles were identified whilst the tbpBA operon was represented by 28 unique alleles that could be assigned to ..
TTR familial amyloid polyneuropathy: does a mitochondrial polymorphism entirely explain the parent-of-origin difference in penetrance?Bernard Bonaïti
INRA GABI, Jouy en Josas, France
Eur J Hum Genet 18:948-52. 2010The Val30Met transthyretin familial amyloid polyneuropathy (TTR-V30M-FAP) is the most frequent familial amyloidosis, with autosomal dominant transmission. This severe disease shows important differences in age of onset and penetrance...
Inhibition by lead of production and secretion of transthyretin in the choroid plexus: its relation to thyroxine transport at blood-CSF barrierW Zheng
School of Public Health, Department of Pharmacology, College of Physicians and Surgeons, Columbia University, New York, NY 10032, USA
Toxicol Appl Pharmacol 155:24-31. 1999Long-term, low-dose Pb exposure in rats is associated with a significant decrease in transthyretin (TTR) concentrations in the CSF...
Identification of TbpA residues required for transferrin-iron utilization by Neisseria gonorrhoeaeJennifer M Noto
Department of Microbiology and Immunology, Virginia Commonwealth University Medical Center, Richmond, VA 23298 0678, USA
Infect Immun 76:1960-9. 2008..The gonococcal transferrin-iron uptake system is composed of two transferrin binding proteins, TbpA and TbpB...
Detection and subtyping of Actinobacillus pleuropneumoniae strains by PCR-RFLP analysis of the tbpA and tbpB genesV A de la Puente Redondo
Section of Microbiology and Immunology, Department of Animal Health, Faculty of Veterinary Medicine, , Spain
Res Microbiol 151:669-81. 2000..The A. pleuropneumoniae tbpA and tbpB genes were used as targets for amplification of DNA fragments, with a pair of specific primers for each ..
Thyroxine binding in a TTR Met 119 kindredI L Alves
Centro de Estudos de Paramiloidose, Hospital de Santo Antonio, Porto, Portugal
J Clin Endocrinol Metab 77:484-8. 1993Recently, a transthyretin variant, TTR Met 119, in which methionine substitutes for threonine 119, a component of the protein's iodothyronine binding site, was identified in individuals with transient euthyroid hyperthyroxinemia...
A new transthyretin variant (Glu61Gly) associated with cardiomyopathyMichael Rosenzweig
Amyloid Treatment and Research Program, Boston University School of Medicine, MA 02118, USA
Amyloid 14:65-71. 2007We report the identification of a new transthyretin (TTR) gene mutation and variant protein, Glu61Gly, in a 55-year-old man with progressive cardiomyopathy, mild peripheral neuropathy and bilateral carpal tunnel syndrome...
Secondary glaucoma in patients with familial amyloidotic polyneuropathyAkira Kimura
Department of Ophthalmology, Kumamoto University School of Medicine, 1 1 1 Honjo, Kumamoto 860 8556, Japan
Arch Ophthalmol 121:351-6. 2003To elucidate the clinical features and surgical outcomes of the treatment of secondary glaucoma associated with transthyretin (TTR)-related familial amyloidotic polyneuropathy (FAP).
Transthyretin, thyroxine, and retinol-binding protein in human cerebrospinal fluid: effect of lead exposureW Zheng
Division of Environmental Health Sciences, School of Public Health, Columbia University, New York, New York 10032, USA
Toxicol Sci 61:107-14. 2001b>Transthyretin (TTR), synthesized by the choroid plexus, is proposed to have a role in transport of thyroid hormones in the brain...
The evolutionary and integrative roles of transthyretin in thyroid hormone homeostasisG Schreiber
Department of Biochemistry and Molecular Biology, The University of Melbourne, Parkville, 3010 Victoria, Australia
J Endocrinol 175:61-73. 2002In larger mammals, thyroid hormone-binding plasma proteins are albumin, transthyretin (TTR) and thyroxine (T4)-binding globulin. They differ characteristically in affinities and release rates for T4 and triiodothyronine (T3)...
Gonococcal genes encoding transferrin-binding proteins A and B are arranged in a bicistronic operon but are subject to differential expressionC Ronpirin
Department of Microbiology and Immunology, Medical College of Virginia, Virginia Commonwealth University, Richmond, Virginia 23298 0678, USA
Infect Immun 69:6336-47. 2001..The receptor involved in transferrin iron acquisition is composed of two distinct transferrin-binding proteins, TbpA and TbpB...
tert-Butylphenylacetylene is a potent mechanism-based inactivator of cytochrome P450 2B4: inhibition of cytochrome P450 catalysis by steric hindranceHaoming Zhang
Department of Pharmacology, The University of Michigan, Ann Arbor, MI 48109 5632, USA
Mol Pharmacol 76:1011-8. 2009We have demonstrated that 4-(tert-butyl)-phenylacetylene (tBPA) is a potent mechanism-based inactivator for cytochrome P450 2B4 (P450 2B4) in the reconstituted system...
Purified meningococcal transferrin-binding protein B interacts with a secondary, strain-specific, binding site in the N-terminal lobe of human transferrinI C Boulton
Metalloprotein Research Group, Division of Biomolecular Sciences, King s College London, Guy s Campus, Guy s Hospital, London SE1 9RT, UK
Biochem J 339:143-9. 1999Neisseria meningitidis, grown in iron-limited conditions, produces two transferrin-binding proteins (TbpA and TbpB) that independently and specifically bind human serum transferrin (hTF) but not bovine serum transferrin (bTF)...
Differential binding of apo and holo human transferrin to meningococci and co-localisation of the transferrin-binding proteins (TbpA and TbpB)N B Powell
Microbial Pathogenicity Research Group, Institute of Infections and Immunity, Queen s Medical Centre, Nottingham
J Med Microbiol 47:257-64. 1998..001). The ability of meningococcal mutants lacking either transferrin-binding protein A (TbpA) or TbpB to discriminate between apo-hTf and holo-hTf was also investigated...
Demonstration and characterization of a specific interaction between gonococcal transferrin binding protein A and TonBChristopher D Kenney
Department of Pathology, Medical College of Virginia Campus, Virginia Commonwealth University, Richmond 23298 0678, USA
J Bacteriol 184:6138-45. 2002..Efficient transferrin-iron acquisition is dependent on the combined action of two proteins, designated TbpA and TbpB...
Structure-based investigation on the binding interaction of hydroxylated polybrominated diphenyl ethers with thyroxine transport proteinsJie Cao
State Key Laboratory of Environmental Chemistry and Ecotoxicology, Research Center for Eco Environmental Sciences, Chinese Academy of Sciences, P O Box 2871, Beijing 100085, China
Toxicology 277:20-8. 2010..Binding affinity of the 14 OH-PBDEs with transthyretin (TTR) and thyroxine-binding globulin (TBG) was measured by competitive fluorescence displacement assay...
Doxycycline disrupts transthyretin amyloid: evidence from studies in a FAP transgenic mice modelI Cardoso
Molecular Neurobiology, IBMC, Porto University, Porto, Portugal
FASEB J 20:234-9. 2006..polyneuropathy is an autosomal dominant disorder mainly characterized by the extracellular deposition of transthyretin, with special involvement of the peripheral nerve...
Molecular cloning, tissue distribution, and developmental expression of lamprey transthyretinsRichard G Manzon
Department of Biology, University of Regina, 3737 Wascana Parkway, Regina, Sask, Canada S4S 0A2
Gen Comp Endocrinol 151:55-65. 2007We isolated and cloned full-length cDNAs of transthyretin (TTR) from 2 genera of lamprey, Petromyzon marinus and Lampetra appendix. These sequences represent the first report of TTR sequences in vertebrates basal to teleost fishes...
Determination of a cut-off value for the molar ratio of retinol-binding protein to transthyretin (RBP:TTR) in Bangladeshi patients with low hepatic vitamin A storesFrancisco J Rosales
Nutrition Department, The Pennsylvania State University, University Park, USA
J Nutr 132:3687-92. 2002The purpose of this study was to determine a cut-off value of the molar ratio of retinol-binding protein to transthyretin (RBP:TTR) to indicate marginal vitamin A (VA) deficiency...
Homology modelling of transferrin-binding protein A from Neisseria meningitidisJonathan S Oakhill
Metalloprotein Research Group, Randall Division of Cell and Molecular Biophysics, King s College London, New Hunt s House, Guy s Campus, London SE1 1UL, UK
Protein Eng Des Sel 18:221-8. 2005..meningitis, obtains transferrin-bound iron by expressing two outer membrane located transferrin-binding proteins, TbpA and TbpB. TbpA is thought to be an integral outer membrane pore that facilitates iron uptake...
ApoA-I cleaved by transthyretin has reduced ability to promote cholesterol efflux and increased amyloidogenicityMárcia Almeida Liz
Molecular Neurobiology Group, Instituto de Biologia Molecular e Celular IBMC, Porto, Portugal
J Lipid Res 48:2385-95. 2007A fraction of plasma transthyretin (TTR) circulates in HDL through binding to apolipoprotein A-I (apoA-I). Moreover, TTR is able to cleave the C terminus of lipid-free apoA-I...
Meningococcal transferrin-binding proteins A and B show cooperation in their binding kinetics for human transferrinRussell H Stokes
Metalloprotein Research Group, Randall Division of Cell and Molecular Biophysics, GKT School of Biomedical Sciences, King s College London, Guy s Campus, New Hunt s House, London SE1 1UL, United Kingdom
Infect Immun 73:944-52. 2005..septicemia, obtains transferrin-bound iron by expressing two outer membrane-located transferrin-binding proteins, TbpA and TbpB. A novel system was developed to investigate the interaction between Tbps and human transferrin...
Use of the retinol-binding protein: transthyretin ratio for assessment of vitamin A status during the acute-phase responseS M Filteau
Centre for International Child Health, Institute of Child Health, London, UK
Br J Nutr 83:513-20. 2000The ratio plasma retinol-binding protein (RBP):transthyretin (TTR) has been proposed as a means to improve the assessment of vitamin A status of individuals with concurrent infection or inflammation...
Insight into the structure and function of the transferrin receptor from Neisseria meningitidis using microcalorimetric techniquesTino Krell
Aventis Pasteur, 1541 Avenue Marcel Merieux, 69280 Marcy l Etoile, France
J Biol Chem 278:14712-22. 2003The transferrin receptor of Neisseria meningitidis is composed of the transmembrane protein TbpA and the outer membrane protein TbpB. Both receptor proteins have the capacity to independently bind their ligand human transferrin (htf)...
Taxanes for the adjuvant treatment of early breast cancer: systematic review and economic evaluationS Ward
School of Health and Related Research ScHARR, University of Sheffield, UK
Health Technol Assess 11:1-144. 2007..To estimate the clinical effectiveness and cost-effectiveness of docetaxel and paclitaxel compared with non-taxane, anthracycline-containing chemotherapy regimens, for the adjuvant treatment of women with early-stage breast cancer...
Regulation of transthyretin by thyroid hormones in fishI Morgado
CCMAR, Universidade do Algarve, Campus de Gambelas, 8005 139 Faro, Portugal
Gen Comp Endocrinol 152:189-97. 2007b>Transthyretin (TTR) is a thyroid hormone-binding protein (THBP) which in its tetrameric form transports thyroid hormones (THs), thyroxine (T(4)) and triiodothyronine (T(3)) in the blood of vertebrates...
Synthesis of transthyretin by the ependymal cells of the subcommissural organH A Montecinos
Facultad de Medicina, Instituto de Histologia y Patologia, Universidad Austral de Chile, Casilla de Correo P O Box 567, Valdivia, Chile
Cell Tissue Res 320:487-99. 2005b>Transthyretin (TTR) is a protein involved in the transport of thyroid hormones in blood and cerebrospinal fluid (CSF). The only known source of brain-produced TTR is the choroid plexus...
Galactose binds to focal segmental glomerulosclerosis permeability factor and inhibits its activityVirginia J Savin
Medical College of Wisconsin, Kidney Disease Center, Milwaukee, WI, USA
Transl Res 151:288-92. 2008Focal segmental glomerulosclerosis (FSGS) is associated with circulating permeability activity (Palb) and recurs after transplantation in about 30% of patients...
Human opsonins induced during meningococcal disease recognize transferrin binding protein complexesA K Lehmann
Institute of Medicine, University of Bergen, N 5021 Bergen, Norway
Infect Immun 67:6526-32. 1999Patient serum opsonins against transferrin binding protein A+B (TbpA+B) complexes from two Neisseria meningitidis strains (K454 and B16B6, with 85- and 68-kDa TbpB, respectively) were quantified by a functional phagocytosis and oxidative ..
Transthyretin regulates thyroid hormone levels in the choroid plexus, but not in the brain parenchyma: study in a transthyretin-null mouse modelJ A Palha
Unidade de Amiloide, Instituto de Biologia Molecular e Celular, Porto, Portugal
Endocrinology 141:3267-72. 2000b>Transthyretin (TTR) is the major T4-binding protein in rodents. Using a TTR-null mouse model we asked the following questions...
Selective binding to transthyretin and tetramer stabilization in serum from patients with familial amyloidotic polyneuropathy by an iodinated diflunisal derivativeMaria Rosário Almeida
Molecular Neurobiology Unit, Instituto de Biologia Molecular e Celular e Departamento de Biologia Molecular, ICBAS Instituto de Ciências Biomédicas Abel Salazar, Universidade do Porto, 4150 180 Porto, Portugal
Biochem J 381:351-6. 2004In familial amyloidotic polyneuropathy, TTR (transthyretin) variants are deposited as amyloid fibrils...
Structural modifications of serum transthyretin in rats during protein-energy malnutritionAndrea Henze
University of Potsdam, Institute of Nutritional Science, Department of Physiology and Pathophysiology, Arthur Scheunert Allee 114 116, D 14558 Nuthetal, Germany
Rapid Commun Mass Spectrom 22:3270-4. 2008b>Transthyretin (TTR) is a sensitive marker of protein-energy malnutrition and changes in serum and expression levels during protein and energy deficiency are well described...
Tear physiology of aqueous deficiency and evaporative dry eyeSantosh Khanal
School of Natural Sciences, University of Western Sydney, New South Wales, Australia
Optom Vis Sci 86:1235-40. 2009..To determine the differences in tear physiology between aqueous deficiency dry eye (ADDE) and evaporative dry eye (EDE), and evaluate their utility in diagnosis...
A genetically engineered purpurin/retinol-binding protein hybrid that binds to transthyretinH Melhus
Department of Cell Research, Uppsala University, Sweden
Biochem Biophys Res Commun 184:938-44. 1992..In contrast to RBP, expressed purpurin did not bind to transthyretin (TTR)...
Kinetic analysis of ligand interaction with the gonococcal transferrin-iron acquisition systemAmanda J DeRocco
Department of Microbiology, Virginia Commonwealth University Medical Center, PO Box 980678, Richmond, VA 23298 0678, USA
Biometals 22:439-51. 2009..b>TbpA is a TonB dependent transporter while TbpB is a lipoprotein that makes iron acquisition from transferrin (Tf) more ..
Characterization of a recombinant transferrin-binding protein A (TbpA) fragment from Haemophilus parasuis serovar 5Sonia Martinez
Departamento de Sanidad Animal, Facultad de Veterinaria, Unidad de Microbiologia e Inmunologia, Universidad de Leon, Leon, Spain
FEMS Microbiol Lett 307:142-50. 2010..This receptor is composed of transferrin-binding protein A (TbpA) and TbpB. As it has been reported for other gram-negative organisms, H...
Receptor-mediated endocytosis of transthyretin by ependymoma cellsS Kuchler-Bopp
Laboratoire de Neurobiologie Moleculaire des Interactions Cellulaires, 11, rue Humann, 67085 Cedex, Strasbourg, France
Brain Res 870:185-94. 2000b>Transthyretin (TTR) is involved in the transport of thyroxine (T4) and retinol-binding protein (RBP) in cerebrospinal fluid (CSF) and serum. TTR is secreted in the CSF by the epithelial cells of choroid plexus...
The transferrin binding protein B of Moraxella catarrhalis elicits bactericidal antibodies and is a potential vaccine antigenL E Myers
Pasteur Merieux Connaught Canada Research, North York, Ontario, Canada M2R 3T4
Infect Immun 66:4183-92. 1998The transferrin binding protein genes (tbpA and tbpB) from two strains of Moraxella catarrhalis have been cloned and sequenced. The genomic organization of the M...
Enhancing functional expression of heterologous lipase B in Escherichia coli by extracellular secretionNiju Narayanan
Department of Chemical Engineering, University of Waterloo, 200 University Avenue West, Waterloo, ON N2L 3G1, Canada
J Ind Microbiol Biotechnol 37:349-61. 2010Functional expression of recombinant Pseudozyma antarctica lipase B (PalB) in Escherichia coli has been technically problematic due to protein misfolding, ineffective disulfide bond formation, and protein instability associated with ..
Upper limb neuropathy such as carpal tunnel syndrome as an initial manifestation of ATTR Val30Met familial amyloid polyneuropathyKana Tojo
Department of Neurology and Rheumatology, Shinshu University School of Medicine, Matsumoto, Japan
Amyloid 17:32-5. 2010We report here two patients with amyloidogenic transthyretin (ATTR) Val30Met familial amyloid polyneuropathy (FAP) who developed numbness in both hands and were diagnosed as having bilateral carpal tunnel syndrome (CTS)...
Poor outcome after liver transplantation for transthyretin amyloid neuropathy in a family with an Ala36Pro transthyretin mutation: case reportKate R Muller
South Australian Liver Transplant Unit, Flinders Medical Centre, Bedford Park, South Australia, Australia
Liver Transpl 16:470-3. 2010b>Transthyretin amyloid polyneuropathy, caused by mutations in the transthyretin gene, is a progressive condition for which liver transplantation is an established treatment...
Destabilization of transthyretin by pathogenic mutations in the DE loopMakoto Takeuchi
Faculty of Pharmaceutical Sciences, University of Toyama, Toyama 930 0194, Japan
Proteins 66:716-25. 2007b>Transthyretin single-amino-acid variants are responsible for familial amyloidotic polyneuropathy, in which transthyretin variants accumulate extracellularly in the form of fibrillar aggregates...
Novel Zn2+-binding sites in human transthyretin: implications for amyloidogenesis and retinol-binding protein recognitionLeonardo de C Palmieri
Instituto de Bioquimica Medica, Universidade Federal do Rio de Janeiro, Rio de Janeiro 21941 590, Brazil
J Biol Chem 285:31731-41. 2010Human transthyretin (TTR) is a homotetrameric protein involved in several amyloidoses. Zn(2+) enhances TTR aggregation in vitro, and is a component of ex vivo TTR amyloid fibrils...
Hepatocyte nuclear factor-4? interacts with other hepatocyte nuclear factors in regulating transthyretin gene expressionZhongyan Wang
Department of Surgery, Boston University School of Medicine, Boston, MA 02118, USA
FEBS J 277:4066-75. 2010b>Transthyretin is a negative acute phase protein whose serum level decreases during the acute phase response...
Targeting of the highly conserved threonine 302 residue of cytochromes P450 2B family during mechanism-based inactivation by aryl acetylenesHaoming Zhang
Department of Pharmacology, The University of Michigan, 1150 W Medical Center Drive, Ann Arbor, MI 48109, USA
Arch Biochem Biophys 507:135-43. 2011..of three mammalian P450s in the 2B family, CYP2B1 (rat), 2B4 (rabbit) and 2B6 (human) by tert-butylphenylacetylene (tBPA)...
The binding of synthetic triiodo l-thyronine analogs to human transthyretin: molecular basis of cooperative and non-cooperative ligand recognitionDaniela B B Trivella
Instituto de Física de São Carlos Universidade de São Paulo, Sao Carlos, SP, Brazil
J Struct Biol 173:323-32. 2011b>Transthyretin (TTR) is a tetrameric ?-sheet-rich transporter protein directly involved in human amyloid diseases...
Structural and functional evolution of transthyretin and transthyretin-like proteinsSarah C Hennebry
Department of Biochemistry and Molecular Biology, The University of Melbourne, 3010 Victoria, Australia
Proteins 64:1024-45. 2006b>Transthyretin (TTR) is a tetrameric protein involved in the distribution of thyroid hormones in vertebrates. The amino acid sequence of TTR is highly conserved across vertebrates...
Thermodynamic stability and denaturation kinetics of a benign natural transthyretin mutant identified in a Danish kindredMinna Groenning
Department of Pharmaceutics and Analytical Chemistry, Faculty of Pharmaceutical Sciences, University of Copenhagen, Denmark
Amyloid 18:35-46. 2011The disease phenotype of transthyretin (TTR) is dramatically influenced by single point mutations in the TTR gene. Herein, we report on a novel mutation D99N (Asp99Asn) in TTR found in a Danish kindred...
Biomarkers of inflammation and malnutrition associated with early death in healthy elderly peopleIsabelle Carriere
INSERM, U888, Montpellier, France
J Am Geriatr Soc 56:840-6. 2008..To determine whether malnutrition and biomarkers of inflammation predict all-cause, cancer, and cardiovascular mortality in healthy elderly subjects...
Identification of a novel ligand binding motif in the transthyretin channelLuis Mauricio T R Lima
Faculdade de Farmacia, Universidade Federal do Rio de Janeiro, RJ 21941 902, Brazil
Bioorg Med Chem 18:100-10. 2010The design of therapeutic compounds targeting transthyretin (TTR) is challenging due to the low specificity of interaction in the hormone binding site...
[Structural changes of transthyretin and its cytotoxicity]Mineyuki Mizuguchi
Graduate School of Medicine and Pharmaceutical Sciences, University of Toyama, 2630 Sugitani, Toyama 930-0194, Japan
Seikagaku 80:751-4. 2008
The modulation of transthyretin tetramer stability by cysteine 10 adducts and the drug diflunisal. Direct analysis by fluorescence-detected analytical ultracentrifugationJonathan S Kingsbury
Department of Biochemistry, Boston University School of Medicine, Boston, Massachusetts 02118, USA
J Biol Chem 283:11887-96. 2008b>Transthyretin (TTR) is normally a stable plasma protein. However, in cases of familial TTR-related amyloidosis and senile systemic amyloidosis (SSA), TTR is deposited as amyloid fibrils, leading to organ dysfunction and possibly death...
Transthyretin forms amyloid fibrils at physiological pH with ultrasonicationYohei Misumi
Department of Neurology, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Japan
Amyloid 15:234-9. 2008In transthyretin (TTR)-related amyloidosis, wild-type TTR (WT-TTR), as well as mutated TTRs play important roles in the pathogenesis of senile systemic amyloidosis and familial amyloidotic polyneuropathy...
Serum levels of variants of transthyretin down-regulation in cholangiocarcinomaLiyun Liu
Key Laboratory for Cell Proliferation and Regulation Biology, Ministry of Education, Beijing Normal University, The People s Republic of China
J Cell Biochem 104:745-55. 2008..Cholangiocarcinoma (CC) is devastating neoplasm and very few specific biomarkers could be used in clinical diagnosis. A study was taken to find novel serum biomarkers for CC...
[Transthyretin: it's miracle function and pathogenesis]Yukio Ando
Department of Diagnostic Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto 860 8556, Japan
Rinsho Byori 57:228-35. 2009b>Transthyretin (TTR) was previously called prealbumin because the band it formed on agarose gel electrophoresis at pH 8.6 was at the prealbumin position...
Binding interactions in early- and late-stage amyloid aggregates of TTR(105-115)Yanfang Liang
York University, Department of Chemistry, Toronto, ON, Canada M3J 1P6
Biophys Chem 144:1-8. 2009..STD) NMR spectroscopy to acquire an atom-specific map of the interactions between soluble and aggregated Transthyretin peptide (TTR(105-115)) in early- and late-stage amyloidogenesis...
Research Grants
- PROBING THE BIOCHEMICAL MECHANISM OF AMYLOID DISEASESJeffery Kelly; Fiscal Year: 2001..In the familial disease, a mutated form transthyretin (prealbumin) (TTR) is deposited as fibrils in peripheral nerves or in specific organs while in the senile form ..
- SOLID STATE NMR STUDIES OF PEPTIDES AND PROTEINSRobert Griffin; Fiscal Year: 2009..1) With ssNMR measurements of intermolecular distances on both the WT TTR and L111M mutant peptides from transthyretin we plan to refine the structures of the amyloid protofilaments formed by these two peptides...
- SOLID STATE NMR STUDIES OF PEPTIDES AND PROTEINSROBERT GUY GRIFFIN; Fiscal Year: 2010..1) With ssNMR measurements of intermolecular distances on both the WT TTR and L111M mutant peptides from transthyretin we plan to refine the structures of the amyloid protofilaments formed by these two peptides...
- Transthyretin's Regulatory Role in Beta-Amyloid Aggregation and ToxicityREGINA MURPHY; Fiscal Year: 2009b>Transthyretin's Regulatory Role in Beta-Amyloid Aggregation and Toxicity Alzheimer's disease (AD) has been linked to deposition of beta-amyloid (A) as amyloid plaques in the brain...
- Transthyretin's Regulatory Role in Beta-Amyloid Aggregation and ToxicityREGINA M contact MURPHY; Fiscal Year: 2010b>Transthyretin's Regulatory Role in Beta-Amyloid Aggregation and Toxicity Alzheimer's disease (AD) has been linked to deposition of beta-amyloid (A) as amyloid plaques in the brain...
- Immunoglobulin Structure, Stability and AgingYair Argon; Fiscal Year: 2010....
- Immunoglobulin Structure, Stability and AgingYair Argon; Fiscal Year: 2007....
- PATHOGENESIS OF HEREDITARY TRANSTHYRETIN AMYLOIDOSISMerrill Benson; Fiscal Year: 2003..abstract): The overall objective of this proposal is to define the pathophysiology of the autosomal dominant transthyretin amyloidoses...
- PATHOGENESIS OF HEREDITARY TRANSTHYRETIN AMYLOIDOSISMerrill Benson; Fiscal Year: 1999The overall objective of this proposal is to define the pathophysiology of the autosomal dominant transthyretin amyloidoses...
- Cardiac Amyloidosis in Aging African AmericansJoel Buxbaum; Fiscal Year: 2005..form of late-onset amyloidosis due to a substitution of ILE for VAL at position 122 in the serum protein transthyretin (TTR)...
- Cardiac Amyloidosis in Aging African AmericanJoel N Buxbaum; Fiscal Year: 2010..form of late-onset amyloidosis due to a substitution of ILE for VAL at position 122 in the serum protein transthyretin (TTR) contributes to this racial disparity...
- CNS DEVELOPMENT UNDER HYPERGRAVITY : MODULATION BY TH?ELIZABETH SAJDEL SULKOWSKA; Fiscal Year: 2004..These studies will aid in developing an understanding of how altered gravity affects CNS development, what role the thyroid hormone plays in that response, and whether this response can be modulated by hormonal therapy. ..
- CHOROID PLEXUS AS A TARGET IN LEAD INDUCED NEUROTOXICITYWei Zheng; Fiscal Year: 2000..Recently, we demonstrated that deposition of Pb in CP is associated with a significant decrease in CSF transthyretin concentration...
- Effect of diflunisal (IND68092) on familial amyloidosisJOHN BERK; Fiscal Year: 2009Familial amyloidotic polyneuropathy (FAP) is a lethal autosomal dominant disease mediated by misfolding of transthyretin, a transport protein produced by the liver...
- Improving Depression Treatment Outcomes with an Insulin-Sensitizing AgentPatrick J Lustman; Fiscal Year: 2010..The findings would represent a significant advance in depression treatment. ..
- Improving Depression Treatment Outcomes with an Insulin-Sensitizing AgentPatrick Lustman; Fiscal Year: 2007..The findings would represent a significant advance in depression treatment. ..
- Improving Depression Treatment Outcomes with an Insulin-Sensitizing AgentPatrick Lustman; Fiscal Year: 2009..The findings would represent a significant advance in depression treatment. ..
- Abcb11 and Hepatic TransportAnne Henkel; Fiscal Year: 2007..We will characterize the role of bile salts in regulating gene and protein expression in the TTR-Abcb11 model as a means to establish the mechanism by which Abcbl 1 predisposes to cholelithiasis and hepatic steatosis. ..
