메뉴 건너뛰기




Volumn 36, Issue 3, 2013, Pages 513-523

Non-physiological amino acid (NPAA) therapy targeting brain phenylalanine reduction: Pilot studies in PAH ENU2 mice

Author keywords

[No Author keywords available]

Indexed keywords

2 AMINO 2 METHYLPROPIONIC ACID; 2 AMINOBICYCLO[2.2.1]HEPTANE 2 CARBOXYLIC ACID; 2 METHYL 2 METHYLAMINOPROPIONIC ACID; ALANINE TRANSPORTER; AMINO ACID TRANSPORTER; BRANCHED CHAIN AMINO ACID; METHIONINE; NORLEUCINE; PHENYLALANINE; TYROSINE; UNCLASSIFIED DRUG;

EID: 84877772598     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-012-9524-8     Document Type: Article
Times cited : (15)

References (40)
  • 1
    • 0344853437 scopus 로고
    • Bacterial metabolism of 2-methylalanine
    • 14234784 1:CAS:528:DyaF2MXktlCq
    • Aaslestad HG, Larson AD (1964) Bacterial metabolism of 2-methylalanine. J Bacteriol 88:1296-1303
    • (1964) J Bacteriol , vol.88 , pp. 1296-1303
    • Aaslestad, H.G.1    Larson, A.D.2
  • 2
    • 70349298552 scopus 로고    scopus 로고
    • Metabolic profiling in phenylalanine hydroxylase-deficient (Pah-/-) mouse brain reveals decreased amino acid neurotransmitters and preferential alterations of the serotoninergic system
    • Arning E, Bottiglieri T, Sun Q et al (2009) Metabolic profiling in phenylalanine hydroxylase-deficient (Pah-/-) mouse brain reveals decreased amino acid neurotransmitters and preferential alterations of the serotoninergic system. Mol Genet Metab 98:21
    • (2009) Molec Genet Metab , vol.98 , pp. 21
    • Arning, E.1    Bottiglieri, T.2    Sun, Q.3
  • 3
    • 0242321838 scopus 로고    scopus 로고
    • Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters
    • 12930836 10.1074/jbc.M305221200 1:CAS:528:DC%2BD3sXosFSrtL4%3D
    • Babu E, Kanai Y, Chairoungdua A et al (2003) Identification of a novel system L amino acid transporter structurally distinct from heterodimeric amino acid transporters. J Biol Chem 278:43838-43845
    • (2003) J Biol Chem , vol.278 , pp. 43838-43845
    • Babu, E.1    Kanai, Y.2    Chairoungdua, A.3
  • 4
    • 15744398433 scopus 로고    scopus 로고
    • Identification of LAT4, a novel amino acid transporter with system L activity
    • 15659399 10.1074/jbc.M408638200 1:CAS:528:DC%2BD2MXisVyju7Y%3D
    • Bodoy S, Martín L, Zorzano A, Palacín M, Estévez R, Bertran J (2005) Identification of LAT4, a novel amino acid transporter with system L activity. J Biol Chem 280:12002-12011
    • (2005) J Biol Chem , vol.280 , pp. 12002-12011
    • Bodoy, S.1    Martín, L.2    Zorzano, A.3    Palacín, M.4    Estévez, R.5    Bertran, J.6
  • 5
    • 0038469628 scopus 로고    scopus 로고
    • Transfer of glutamine between astrocytes and neurons
    • 11331400 10.1046/j.1471-4159.2001.00322.x
    • Bröer S, Brookes N (2001) Transfer of glutamine between astrocytes and neurons. J Neurochem 77:705-719
    • (2001) J Neurochem , vol.77 , pp. 705-719
    • Bröer, S.1    Brookes, N.2
  • 6
    • 0022966985 scopus 로고
    • Phenylalanine transport at the human blood-brain barrier. Studies with isolated human brain capillaries
    • 3700406 1:CAS:528:DyaL28Xit1anu7o%3D
    • Choi TB, Pardridge WM (1986) Phenylalanine transport at the human blood-brain barrier. Studies with isolated human brain capillaries. J Biol Chem 261:6536-6541
    • (1986) J Biol Chem , vol.261 , pp. 6536-6541
    • Choi, T.B.1    Pardridge, W.M.2
  • 7
    • 0038821390 scopus 로고
    • Effects of feeding individual amino acids upon the distribution of other amino acids between cells and extracellular fluid
    • 18901170 1:CAS:528:DyaH1cXhslOisw%3D%3D
    • Christensen HN, Streicher JA, Elbinger RL (1948) Effects of feeding individual amino acids upon the distribution of other amino acids between cells and extracellular fluid. J Biol Chem 172:515-524
    • (1948) J Biol Chem , vol.172 , pp. 515-524
    • Christensen, H.N.1    Streicher, J.A.2    Elbinger, R.L.3
  • 8
    • 0025095302 scopus 로고
    • Role of amino acid transport and countertransport in nutrition and metabolism
    • 2404290 1:CAS:528:DyaK3cXpsF2hsQ%3D%3D
    • Christensen HN (1990) Role of amino acid transport and countertransport in nutrition and metabolism. Physiol Rev 70:43-77
    • (1990) Physiol Rev , vol.70 , pp. 43-77
    • Christensen, H.N.1
  • 9
    • 60049101293 scopus 로고    scopus 로고
    • LAT-1 expression in pre- and post-implantation embryos and placenta
    • 19193433 10.1016/j.placenta.2008.12.015 1:CAS:528:DC%2BD1MXhvF2ku78%3D
    • Chrostowski MK, McGonnigal BG, Stabila JP, Padbury JF (2009) LAT-1 expression in pre- and post-implantation embryos and placenta. Placenta 30:270-276
    • (2009) Placenta , vol.30 , pp. 270-276
    • Chrostowski, M.K.1    McGonnigal, B.G.2    Stabila, J.P.3    Padbury, J.F.4
  • 10
    • 0015805045 scopus 로고
    • Transport of methionine and proline by rat liver slices and the effect of certain hormones
    • 4359105 1:CAS:528:DyaE2cXht12qtA%3D%3D
    • Crawhall JC, Purkiss P (1973) Transport of methionine and proline by rat liver slices and the effect of certain hormones. Biochem J 136:15-24
    • (1973) Biochem J , vol.136 , pp. 15-24
    • Crawhall, J.C.1    Purkiss, P.2
  • 11
    • 0023658398 scopus 로고
    • Candida L-norleucine, leucine:2-oxoglutarate aminotransferase. Purification and properties
    • 10.1111/j.1432-1033.1987.tb13315.x
    • Der Garabedian PA, Vermeersch JJ (1987) Candida L-norleucine, leucine:2-oxoglutarate aminotransferase. Purification and properties. Eur J Biochem 167:141-147
    • (1987) Eur J Biochem , vol.167 , pp. 141-147
    • Der Garabedian, P.A.1    Vermeersch, J.J.2
  • 12
    • 0029072351 scopus 로고
    • Nutritional value of essential amino acids in the treatment of adults with phenylketonuria
    • 7564226 10.1007/BF00711746 1:STN:280:DyaK28%2Flslentg%3D%3D
    • Dotremont H, Francois B, Diels M, Gillis P (1995) Nutritional value of essential amino acids in the treatment of adults with phenylketonuria. J Inherit Metab Dis 18:127-130
    • (1995) J Inherit Metab Dis , vol.18 , pp. 127-130
    • Dotremont, H.1    Francois, B.2    Diels, M.3    Gillis, P.4
  • 13
    • 0028178476 scopus 로고
    • Transport of alpha-aminoisobutyric acid across the blood-brain barrier studied with in situ perfusion of rat brain
    • 8032907 10.1016/0006-8993(94)90014-0 1:CAS:528:DyaK2cXis1Krsb4%3D
    • Ennis SR, Ren XD, Betz AL (1994) Transport of alpha-aminoisobutyric acid across the blood-brain barrier studied with in situ perfusion of rat brain. Brain Res 643:100-107
    • (1994) Brain Res , vol.643 , pp. 100-107
    • Ennis, S.R.1    Ren, X.D.2    Betz, A.L.3
  • 14
    • 77957237994 scopus 로고    scopus 로고
    • Suboptimal outcomes in patients with PKU treated early with diet alone: Revisiting the evidence
    • 20678948 10.1016/j.ymgme.2010.05.017 1:CAS:528:DC%2BC3cXht1CmtbjN
    • Enns GM, Koch R, Brumm V, Blakely E, Suter R, Jurecki E (2010) Suboptimal outcomes in patients with PKU treated early with diet alone: revisiting the evidence. Mol Genet Metab 101:99-109
    • (2010) Mol Genet Metab , vol.101 , pp. 99-109
    • Enns, G.M.1    Koch, R.2    Brumm, V.3    Blakely, E.4    Suter, R.5    Jurecki, E.6
  • 15
    • 80052184853 scopus 로고    scopus 로고
    • Role of serotonin in Alzheimer's disease: A new therapeutic target
    • 21870888 10.2165/11590190-000000000-00000 1:CAS:528:DC%2BC3MXhsVWjsbrK
    • Geldenhuys WJ, Van der Schyf CJ (2011) Role of serotonin in Alzheimer's disease: a new therapeutic target. CNS Drugs 25:765-781
    • (2011) CNS Drugs , vol.25 , pp. 765-781
    • Geldenhuys, W.J.1    Van Der Schyf, C.J.2
  • 16
    • 84863271896 scopus 로고    scopus 로고
    • β-Cell-protective effect of 2-aminobicyclo-(2,1,1)-heptane-2- carboxylic acid as a glutamate dehydrogenase activator in db/db mice
    • Han SJ, Choi S-E, Yi S-A et al (2012) β-Cell-protective effect of 2-aminobicyclo-(2,1,1)-heptane-2-carboxylic acid as a glutamate dehydrogenase activator in db/db mice. J Endocrinol 212:307-315
    • (2012) J Endocrinol , vol.212 , pp. 307-315
    • Han, S.J.1    Choi, S.-E.2    Yi, S.-A.3
  • 17
    • 0032508585 scopus 로고    scopus 로고
    • Expression cloning and characterization of a transporter for large neutral amino acids activated by the heavy chain of 4F2 antigen (CD98)
    • 9726963 10.1074/jbc.273.37.23629 1:CAS:528:DyaK1cXmtFOktLo%3D
    • Kanai Y, Segawa H, Miyamoto K, Uchino H, Takeda E, Endou H (1998) Expression cloning and characterization of a transporter for large neutral amino acids activated by the heavy chain of 4F2 antigen (CD98). J Biol Chem 273:23629-23632
    • (1998) J Biol Chem , vol.273 , pp. 23629-23632
    • Kanai, Y.1    Segawa, H.2    Miyamoto, K.3    Uchino, H.4    Takeda, E.5    Endou, H.6
  • 18
    • 0029588231 scopus 로고
    • Blood-brain barrier transport of amino acids in healthy controls and in patients with phenylketonuria
    • 8750601 10.1007/BF02436753 1:STN:280:DyaK28zktlensA%3D%3D
    • Knudsen GM, Hasselbalch S, Toft PB, Christensen E, Paulson OB, Lou H (1995) Blood-brain barrier transport of amino acids in healthy controls and in patients with phenylketonuria. J Inherit Metab Dis 18:653-664
    • (1995) J Inherit Metab Dis , vol.18 , pp. 653-664
    • Knudsen, G.M.1    Hasselbalch, S.2    Toft, P.B.3    Christensen, E.4    Paulson, O.B.5    Lou, H.6
  • 19
    • 79960352475 scopus 로고    scopus 로고
    • The monoamine neurotransmitter disorders: An expanding range of neurological syndromes
    • 21777827 10.1016/S1474-4422(11)70141-7 1:CAS:528:DC%2BC3MXptlyqsb4%3D
    • Kurian MA, Gissen P, Smith M, Heales S Jr, Clayton PT (2011) The monoamine neurotransmitter disorders: an expanding range of neurological syndromes. Lancet Neurol 10:721-733
    • (2011) Lancet Neurol , vol.10 , pp. 721-733
    • Kurian, M.A.1    Gissen, P.2    Smith, M.3    Heales, Jr.S.4    Clayton, P.T.5
  • 20
    • 0020557142 scopus 로고
    • Characteristics of an uptake system for alpha-aminoisobutyric acid in Leishmania tropica promastigotes
    • 6864594 1:CAS:528:DyaL3sXktF2ns74%3D
    • Lepley PR, Mukkada AJ (1983) Characteristics of an uptake system for alpha-aminoisobutyric acid in Leishmania tropica promastigotes. J Protozool 30:41-46
    • (1983) J Protozool , vol.30 , pp. 41-46
    • Lepley, P.R.1    Mukkada, A.J.2
  • 21
    • 12144287436 scopus 로고    scopus 로고
    • L-type amino acid transporter-1 overexpression and melphalan sensitivity in Barrett's adenocarcinoma
    • 15068672 10.1593/neo.04205 1:CAS:528:DC%2BD2cXjslSgt7s%3D
    • Lin J, Raoof DA, Thomas DG et al (2004) L-type amino acid transporter-1 overexpression and melphalan sensitivity in Barrett's adenocarcinoma. Neoplasia 6:74-84
    • (2004) Neoplasia , vol.6 , pp. 74-84
    • Lin, J.1    Raoof, D.A.2    Thomas, D.G.3
  • 22
    • 33751029864 scopus 로고    scopus 로고
    • Large neutral amino acids in the treatment of phenylketonuria (PKU)
    • 16988900 10.1007/s10545-006-0395-8 1:CAS:528:DC%2BD28Xht1SgurfM
    • Matalon R, Michals-Matalon K, Bhatia G et al (2006) Large neutral amino acids in the treatment of phenylketonuria (PKU). J Inherit Metab Dis 29:732-738
    • (2006) J Inherit Metab Dis , vol.29 , pp. 732-738
    • Matalon, R.1    Michals-Matalon, K.2    Bhatia, G.3
  • 23
    • 0014265167 scopus 로고
    • The influence of high phenylalanine and tyrosine on the concentrations of essential amino acids in brain
    • 5638620 10.1111/j.1471-4159.1968.tb06202.x 1:CAS:528:DyaF1cXns1Wqsg%3D%3D
    • McKean CM, Boggs DE, Peterson NA (1968) The influence of high phenylalanine and tyrosine on the concentrations of essential amino acids in brain. J Neurochem 15:235-241
    • (1968) J Neurochem , vol.15 , pp. 235-241
    • McKean, C.M.1    Boggs, D.E.2    Peterson, N.A.3
  • 25
    • 33646161224 scopus 로고    scopus 로고
    • Prostaglandin J2 reduces catechol-O-methyltransferase activity and enhances dopamine toxicity in neuronal cells
    • 16406650 10.1016/j.nbd.2005.11.006 1:CAS:528:DC%2BD28XktVyjsrs%3D
    • Ogburn KD, Bottiglieri T, Wang Z, Figueiredo-Pereira ME (2006) Prostaglandin J2 reduces catechol-O-methyltransferase activity and enhances dopamine toxicity in neuronal cells. Neurobiol Dis 22:294-301
    • (2006) Neurobiol Dis , vol.22 , pp. 294-301
    • Ogburn, K.D.1    Bottiglieri, T.2    Wang, Z.3    Figueiredo-Pereira, M.E.4
  • 26
    • 0033560647 scopus 로고    scopus 로고
    • Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria
    • 10207169 10.1172/JCI5017 1:CAS:528:DyaK1MXis1Gnsbs%3D
    • Pietz J, Kreis R, Rupp A, Mayatepek E, Rating D, Boesch C, Bremer HJ (1999) Large neutral amino acids block phenylalanine transport into brain tissue in patients with phenylketonuria. J Clin Invest 103:1169-1178
    • (1999) J Clin Invest , vol.103 , pp. 1169-1178
    • Pietz, J.1    Kreis, R.2    Rupp, A.3    Mayatepek, E.4    Rating, D.5    Boesch, C.6    Bremer, H.J.7
  • 27
    • 0023176850 scopus 로고
    • Characterization of carrier-mediated transport systems for small neutral amino acids in human fibroblast lysosomes
    • 3106350 1:CAS:528:DyaL2sXktVaktLw%3D
    • Pisoni RL, Flickinger KS, Thoene JG, Christensen HN (1987) Characterization of carrier-mediated transport systems for small neutral amino acids in human fibroblast lysosomes. J Biol Chem 262:6010-6017
    • (1987) J Biol Chem , vol.262 , pp. 6010-6017
    • Pisoni, R.L.1    Flickinger, K.S.2    Thoene, J.G.3    Christensen, H.N.4
  • 28
    • 0033538560 scopus 로고    scopus 로고
    • Identification and functional characterization of a Na + -independent neutral amino acid transporter with broad substrate selectivity
    • 10391916 10.1074/jbc.274.28.19745 1:CAS:528:DyaK1MXksFKmtLk%3D
    • Segawa H, Fukasawa Y, Miyamoto K, Takeda E, Endou H, Kanai Y (1999) Identification and functional characterization of a Na + -independent neutral amino acid transporter with broad substrate selectivity. J Biol Chem 274:19745-19751
    • (1999) J Biol Chem , vol.274 , pp. 19745-19751
    • Segawa, H.1    Fukasawa, Y.2    Miyamoto, K.3    Takeda, E.4    Endou, H.5    Kanai, Y.6
  • 29
    • 0028372195 scopus 로고
    • Characteristics of alpha-aminoisobutyric acid transport by lactating rat mammary gland
    • 8188948 10.1017/S0022029900028016
    • Shennan DB, McNeillie SA (1994) Characteristics of alpha-aminoisobutyric acid transport by lactating rat mammary gland. J Dairy Res 61:9-19
    • (1994) J Dairy Res , vol.61 , pp. 9-19
    • Shennan, D.B.1    McNeillie, S.A.2
  • 30
    • 70349276673 scopus 로고    scopus 로고
    • Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD)
    • 19699299 10.1016/j.bbadis.2009.08.006 1:CAS:528:DC%2BD1MXhtF2jsr3M
    • Skvorak KJ, Hager EJ, Arning E et al (2009) Hepatocyte transplantation (HTx) corrects selected neurometabolic abnormalities in murine intermediate maple syrup urine disease (iMSUD). Biochim Biophys Acta 1792:1004-1010
    • (2009) Biochim Biophys Acta , vol.1792 , pp. 1004-1010
    • Skvorak, K.J.1    Hager, E.J.2    Arning, E.3
  • 31
    • 0023634699 scopus 로고
    • Kinetics of neutral amino acid transport across the blood-brain barrier
    • 3668544 10.1111/j.1471-4159.1987.tb01039.x 1:CAS:528:DyaL2sXmtFGhu70%3D
    • Smith QR, Momma S, Aoyagi M, Rapoport SI (1987) Kinetics of neutral amino acid transport across the blood-brain barrier. J Neurochem 49:1651-1658
    • (1987) J Neurochem , vol.49 , pp. 1651-1658
    • Smith, Q.R.1    Momma, S.2    Aoyagi, M.3    Rapoport, S.I.4
  • 32
    • 0022508238 scopus 로고
    • Tissue amino acids in rats fed norleucine, norvaline, homoarginine or other amino acid analogues
    • 2428957 1:CAS:528:DyaL28XlvV2ruro%3D
    • Tews JK, Harper AE (1986) Tissue amino acids in rats fed norleucine, norvaline, homoarginine or other amino acid analogues. J Nutr 116:1464-1472
    • (1986) J Nutr , vol.116 , pp. 1464-1472
    • Tews, J.K.1    Harper, A.E.2
  • 33
    • 0025318133 scopus 로고
    • Norleucine: A branched-chain amino acid analog affecting feeding behavior of rats
    • 2345765 10.1016/0091-3057(90)90379-V 1:CAS:528:DyaK3cXitFGmsb8%3D
    • Tews JK, Repa JJ, Harper AE (1990) Norleucine: a branched-chain amino acid analog affecting feeding behavior of rats. Pharmacol Biochem Behav 35:911-921
    • (1990) Pharmacol Biochem Behav , vol.35 , pp. 911-921
    • Tews, J.K.1    Repa, J.J.2    Harper, A.E.3
  • 34
    • 0025854573 scopus 로고
    • Branched-chain and other amino acids in tissues of rats fed leucine-limiting amino acid diets containing norleucine
    • 2002408 1:CAS:528:DyaK3MXit1Orsbk%3D
    • Tews JK, Repa JJ, Harper AE (1991) Branched-chain and other amino acids in tissues of rats fed leucine-limiting amino acid diets containing norleucine. J Nutr 121:364-378
    • (1991) J Nutr , vol.121 , pp. 364-378
    • Tews, J.K.1    Repa, J.J.2    Harper, A.E.3
  • 35
    • 0023805136 scopus 로고
    • Some characteristics of threonine transport across the blood-brain barrier of the rat
    • 3138388 10.1111/j.1471-4159.1988.tb03098.x 1:CAS:528:DyaL1cXmtVyksb0%3D
    • Tovar A, Tews JK, Torres N, Harper AE (1988) Some characteristics of threonine transport across the blood-brain barrier of the rat. J Neurochem 51:1285-1293
    • (1988) J Neurochem , vol.51 , pp. 1285-1293
    • Tovar, A.1    Tews, J.K.2    Torres, N.3    Harper, A.E.4
  • 36
    • 72249098974 scopus 로고    scopus 로고
    • Future treatment strategies in phenylketonuria
    • 20123478 10.1016/j.ymgme.2009.10.008
    • van Spronsen FJ, Enns GM (2010) Future treatment strategies in phenylketonuria. Mol Genet Metab 99:S90-S95
    • (2010) Mol Genet Metab , vol.99
    • Van Spronsen, F.J.1    Enns, G.M.2
  • 38
    • 0025324930 scopus 로고
    • Facilitated transport of L-phenylalanine across blood-nerve barrier of rat peripheral nerve
    • 2360693 1:CAS:528:DyaK3cXkslGntrw%3D
    • Wadhwani KC, Smith QR, Rapoport SI (1990) Facilitated transport of L-phenylalanine across blood-nerve barrier of rat peripheral nerve. Am J Physiol 258:R1436-R1444
    • (1990) Am J Physiol , vol.258
    • Wadhwani, K.C.1    Smith, Q.R.2    Rapoport, S.I.3
  • 39
    • 0033565404 scopus 로고    scopus 로고
    • Cognitive deficits in a genetic mouse model of the most common biochemical cause of human mental retardation
    • 10407053 1:CAS:528:DyaK1MXksVyku7o%3D
    • Zagreda L, Goodman J, Druin DP, McDonald D, Diamond A (1999) Cognitive deficits in a genetic mouse model of the most common biochemical cause of human mental retardation. J Neurosci 19:6175-6182
    • (1999) J Neurosci , vol.19 , pp. 6175-6182
    • Zagreda, L.1    Goodman, J.2    Druin, D.P.3    McDonald, D.4    Diamond, A.5
  • 40
    • 64449086258 scopus 로고    scopus 로고
    • Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease
    • 19293241 10.1093/brain/awp024
    • Zinnanti WJ, Lazovic J, Griffin K et al (2009) Dual mechanism of brain injury and novel treatment strategy in maple syrup urine disease. Brain 132:903-918
    • (2009) Brain , vol.132 , pp. 903-918
    • Zinnanti, W.J.1    Lazovic, J.2    Griffin, K.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.