메뉴 건너뛰기




Volumn 33, Issue 2, 2010, Pages 121-127

Docosahexaenoic acid status in females of reproductive age with maple syrup urine disease

Author keywords

[No Author keywords available]

Indexed keywords

DOCOSAHEXAENOIC ACID; FATTY ACID; LINOLENIC ACID;

EID: 77953228971     PISSN: 01418955     EISSN: 15732665     Source Type: Journal    
DOI: 10.1007/s10545-010-9066-x     Document Type: Article
Times cited : (9)

References (41)
  • 1
    • 0028947747 scopus 로고
    • Fatty acid composition of erythrocyte, platelet, and serum lipids in strict vegans
    • 10.1007/BF02536047 1:CAS:528:DyaK2MXltFCqurw%3D 7609607
    • JJ Agren ML Törmälä MT Nenonen OO Hänninen 1995 Fatty acid composition of erythrocyte, platelet, and serum lipids in strict vegans Lipids 30 4 365 369 10.1007/BF02536047 1:CAS:528:DyaK2MXltFCqurw%3D 7609607
    • (1995) Lipids , vol.30 , Issue.4 , pp. 365-369
    • Agren, J.J.1    Törmälä, M.L.2    Nenonen, M.T.3    Hänninen, O.O.4
  • 3
    • 51749101297 scopus 로고    scopus 로고
    • Flaxseed oil and fish-oil capsule consumption alters human red blood cell n-3 fatty acid composition: A multiple-dosing trial comparing 2 sources of n-3 fatty acid
    • 1:CAS:528:DC%2BD1cXhtFOisb%2FM 18779299
    • G Barcelo-Coblijn EJ Murphy R Othman, et al. 2008 Flaxseed oil and fish-oil capsule consumption alters human red blood cell n-3 fatty acid composition: a multiple-dosing trial comparing 2 sources of n-3 fatty acid Am J Clin Nutr 88 3 801 809 1:CAS:528:DC%2BD1cXhtFOisb%2FM 18779299
    • (2008) Am J Clin Nutr , vol.88 , Issue.3 , pp. 801-809
    • Barcelo-Coblijn, G.1    Murphy, E.J.2    Othman, R.3
  • 5
    • 33747889032 scopus 로고    scopus 로고
    • Metabolism of α-linolenic acid in humans
    • DOI 10.1016/j.plefa.2006.05.013, PII S0952327806000834, Proceedings on the 7th fatty acid and cell signalling (FACS) workshop
    • GC Burdge 2006 Metabolism of α-linolenic acid in humans Prostaglandins Leukot Essent Fatty Acids 75 161 168 10.1016/j.plefa.2006.05.013 1:CAS:528:DC%2BD28XptFChtro%3D 16828546 (Pubitemid 44292520)
    • (2006) Prostaglandins Leukotrienes and Essential Fatty Acids , vol.75 , Issue.3 , pp. 161-168
    • Burdge, G.C.1
  • 6
    • 27244445146 scopus 로고    scopus 로고
    • Conversion of α-linolenic acid to longer-chain polyunsaturated fatty acids in human adults
    • DOI 10.1051/rnd:2005047
    • GC Burdge PC Calder 2005 Conversion of α-linolenic acid to longer-chain polyunsaturated fatty acids in human adults Reprod Nutr Dev 45 581 597 10.1051/rnd:2005047 1:CAS:528:DC%2BD2MXht1CmurbJ 16188209 (Pubitemid 41511883)
    • (2005) Reproduction Nutrition Development , vol.45 , Issue.5 , pp. 581-597
    • Burdge, G.C.1    Calder, P.C.2
  • 7
    • 0002977005 scopus 로고    scopus 로고
    • Maple syrup urine disease (branched-chain ketoaciduria)
    • C.R. Scriver A.L. Beaudet W.S. Sly D. Valle (eds). 8 McGraw-Hill New York
    • Chuang DT, Shih VE (2001) Maple syrup urine disease (branched-chain ketoaciduria). In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic and molecular bases of inherited disease, vol. II, 8th edn. McGraw-Hill, New York, pp 1971-2005
    • (2001) The Metabolic and Molecular Bases of Inherited Disease, Vol. II , pp. 1971-2005
    • Chuang, D.T.1    Shih, V.E.2
  • 8
    • 0025265676 scopus 로고
    • Dietary effects on brain fatty acid composition: The reversibility of n-3 fatty acid deficiency and turnover of docosahexanoic acid in the brain, erythrocytes, and plasma of rhesus monkeys
    • 1:CAS:528:DyaK3cXhslOgu7o%3D 2139096
    • WE Connor M Neuringer DS Lin 1990 Dietary effects on brain fatty acid composition: the reversibility of n-3 fatty acid deficiency and turnover of docosahexanoic acid in the brain, erythrocytes, and plasma of rhesus monkeys J Lipid Res 31 237 247 1:CAS:528:DyaK3cXhslOgu7o%3D 2139096
    • (1990) J Lipid Res , vol.31 , pp. 237-247
    • Connor, W.E.1    Neuringer, M.2    Lin, D.S.3
  • 9
    • 0033000315 scopus 로고    scopus 로고
    • Evidence for the unique function of docosahexaenoic acid (DHA) during the evolution of the modern hominid brain
    • 10.1007/BF02562227
    • MA Crawford M Bloom CL Broadhurst, et al. 2000 Evidence for the unique function of docosahexaenoic acid (DHA) during the evolution of the modern hominid brain Lipids 34 S39 S47 10.1007/BF02562227
    • (2000) Lipids , vol.34
    • Crawford, M.A.1    Bloom, M.2    Broadhurst, C.L.3
  • 10
    • 0025940476 scopus 로고
    • Polyunsaturated fatty acid status in patients with phenylketonuria during dietary intervention
    • 10.1016/S0022-3476(05)82405-9 1:STN:280:DyaK38%2FhsVGjtw%3D%3D
    • C Galli C Agostoni C Mosconi 1991 Polyunsaturated fatty acid status in patients with phenylketonuria during dietary intervention J Pedtr 119 4 562 567 10.1016/S0022-3476(05)82405-9 1:STN:280:DyaK38%2FhsVGjtw%3D%3D
    • (1991) J Pedtr , vol.119 , Issue.4 , pp. 562-567
    • Galli, C.1    Agostoni, C.2    Mosconi, C.3
  • 11
    • 25844502497 scopus 로고    scopus 로고
    • Docosahexaenoic acid supplementation in vegetarians effectively increases omega-3 index: A randomized trial
    • DOI 10.1007/s11745-005-1442-9, L9761
    • J Geppert V Kraft H Demmelmair B Koletzko 2005 Docosahexaenoic acid supplementation in vegetarians effectively increases omega-3 index: a randomized trial Lipids 40 8 807 814 10.1007/s11745-005-1442-9 1:CAS:528: DC%2BD2MXhtFSrsL%2FP 16296399 (Pubitemid 41393256)
    • (2005) Lipids , vol.40 , Issue.8 , pp. 807-814
    • Geppert, J.1    Kraft, V.2    Demmelmair, H.3    Koletzko, B.4
  • 12
    • 0030866991 scopus 로고    scopus 로고
    • Effect of increasing breast milk docosahexaenoic acid on plasma and erythrocyte phospholipids fatty acids and neural indices of exclusively breast fed infants
    • 10.1038/sj.ejcn.1600446 1:CAS:528:DyaK1cXjslymtw%3D%3D 9306083
    • RA Gibson MA Neuman M Makrides 1997 Effect of increasing breast milk docosahexaenoic acid on plasma and erythrocyte phospholipids fatty acids and neural indices of exclusively breast fed infants Eur J Clin Nutr 51 578 584 10.1038/sj.ejcn.1600446 1:CAS:528:DyaK1cXjslymtw%3D%3D 9306083
    • (1997) Eur J Clin Nutr , vol.51 , pp. 578-584
    • Gibson, R.A.1    Neuman, M.A.2    Makrides, M.3
  • 13
    • 62549150332 scopus 로고    scopus 로고
    • Quantitation of alpha-linolenic acid elongation to eicosapentaenoic and docosahexaenoic acid as affected by the ratio of n6/n3 fatty acids
    • 10.1186/1743-7075-6-8
    • K Harnack G Andersen V Somoza 2009 Quantitation of alpha-linolenic acid elongation to eicosapentaenoic and docosahexaenoic acid as affected by the ratio of n6/n3 fatty acids Nutr Metab (Lond) 19 6 8 10.1186/1743-7075-6-8
    • (2009) Nutr Metab (Lond) , vol.19 , Issue.6 , pp. 8
    • Harnack, K.1    Andersen, G.2    Somoza, V.3
  • 14
    • 33644827373 scopus 로고    scopus 로고
    • Impact of longitudinal plasma leucine levels on the intellectual outcome in patients with classic MSUD
    • 10.1203/01.pdr.0000190571.60385.34 1:CAS:528:DC%2BD2MXhtlert7zJ 16326996
    • B Hoffmann C Belbling P Shadewaldt 2006 Impact of longitudinal plasma leucine levels on the intellectual outcome in patients with classic MSUD Pediatr Res 59 17 20 10.1203/01.pdr.0000190571.60385.34 1:CAS:528:DC%2BD2MXhtlert7zJ 16326996
    • (2006) Pediatr Res , vol.59 , pp. 17-20
    • Hoffmann, B.1    Belbling, C.2    Shadewaldt, P.3
  • 15
    • 0035715131 scopus 로고    scopus 로고
    • Impaired arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) acid synthesis by phenylalanine metabolites as etiological factors in the neuropathology of phenylketonuria
    • 10.1006/mgme.2001.3148
    • JP Infante VA Huszagh 2004 Impaired arachidonic (20:4n-6) and docosahexaenoic (22:6n-3) acid synthesis by phenylalanine metabolites as etiological factors in the neuropathology of phenylketonuria Mol Genet Metab 72 3 185 198 10.1006/mgme.2001.3148
    • (2004) Mol Genet Metab , vol.72 , Issue.3 , pp. 185-198
    • Infante, J.P.1    Huszagh, V.A.2
  • 16
    • 0035718040 scopus 로고    scopus 로고
    • Quantitative determination of plasma C8-C26 total fatty acids for the biochemical diagnosis of nutritional and metabolic disorders
    • DOI 10.1006/mgme.2001.3170
    • SA Lagerstedt DR Hinrichs SM Batt MJ Magera P Rinaldo JP McConnell 2001 Quantitative determination of plasma C8-C26 total fatty acids for the biochemical diagnosis of nutritional and metabolic disorders Mol Gen Metabol 73 38 45 10.1006/mgme.2001.3170 1:CAS:528:DC%2BD3MXjtlOqt7g%3D (Pubitemid 34177622)
    • (2001) Molecular Genetics and Metabolism , vol.73 , Issue.1 , pp. 38-45
    • Lagerstedt, S.A.1    Hinrichs, D.R.2    Batt, S.M.3    Magera, M.J.4    Rinaldo, P.5    McConnell, J.P.6
  • 17
    • 77953231357 scopus 로고    scopus 로고
    • Relationship between medical food type consumption and plasma polyunsaturated fatty acid status of females of childbearing age with phenylketonuria
    • SH Leatham Yi AB Moser RH Singh 2007 Relationship between medical food type consumption and plasma polyunsaturated fatty acid status of females of childbearing age with phenylketonuria Faseb J 21 688.1
    • (2007) Faseb J , vol.21 , pp. 6881
    • Leatham Yi, S.H.1    Moser, A.B.2    Singh, R.H.3
  • 18
    • 0014830239 scopus 로고
    • Brief clinical and laboratory observations: Folic acid deficiency secondary to a diet for maple syrup urine disease
    • 10.1016/S0022-3476(70)80338-9 1:STN:280:DyaE3c3ktFarsg%3D%3D 4194001
    • HL Levy JT Truman RN Ganz 1970 Brief clinical and laboratory observations: Folic acid deficiency secondary to a diet for maple syrup urine disease J Pediatr 77 294 296 10.1016/S0022-3476(70)80338-9 1:STN:280: DyaE3c3ktFarsg%3D%3D 4194001
    • (1970) J Pediatr , vol.77 , pp. 294-296
    • Levy, H.L.1    Truman, J.T.2    Ganz, R.N.3
  • 20
    • 0032521063 scopus 로고    scopus 로고
    • Dietary therapy for preventing and treating coronary artery disease
    • Masley SC (1998) Dietary therapy for preventing and treating coronary artery disease. Am Fam Physician 57(6):1299-1306
    • (1998) Am Fam Physician , vol.57 , Issue.6 , pp. 1299-1306
    • Masley, S.C.1
  • 22
    • 0001468282 scopus 로고
    • A new syndrome: Progressive familial infantile cerebral dysfunction associated with an unusual urinary substance
    • 1:STN:280:DyaG2M%2Fis1Khug%3D%3D 13214961
    • JH Menkes PL Hurst JM Craig 1954 A new syndrome: progressive familial infantile cerebral dysfunction associated with an unusual urinary substance Pediatrics 14 462 466 1:STN:280:DyaG2M%2Fis1Khug%3D%3D 13214961
    • (1954) Pediatrics , vol.14 , pp. 462-466
    • Menkes, J.H.1    Hurst, P.L.2    Craig, J.M.3
  • 23
    • 0348101019 scopus 로고
    • Cerebral lipids in maple syrup disease
    • 10.1016/S0022-3476(65)80122-6 1:STN:280:DyaF2M%2FmvVymsw%3D%3D
    • JH Menkes M Philippart RE Fiol 1965 Cerebral lipids in maple syrup disease Pediatrics 66 584 594 10.1016/S0022-3476(65)80122-6 1:STN:280: DyaF2M%2FmvVymsw%3D%3D
    • (1965) Pediatrics , vol.66 , pp. 584-594
    • Menkes, J.H.1    Philippart, M.2    Fiol, R.E.3
  • 24
    • 0028868819 scopus 로고
    • Docosahexaenoic acid synthesis in human skin fibroblasts involves peroxisomal retroconversion of tetracosahexaenoic acid
    • 1:CAS:528:DyaK2MXpslGitL0%3D 8656081
    • SA Moore E Hurt E Yoder H Sprecher AA Spector 1995 Docosahexaenoic acid synthesis in human skin fibroblasts involves peroxisomal retroconversion of tetracosahexaenoic acid J Lipid Res 36 11 2433 2443 1:CAS:528:DyaK2MXpslGitL0%3D 8656081
    • (1995) J Lipid Res , vol.36 , Issue.11 , pp. 2433-2443
    • Moore, S.A.1    Hurt, E.2    Yoder, E.3    Sprecher, H.4    Spector, A.A.5
  • 25
    • 0036264313 scopus 로고    scopus 로고
    • Diagnosis and treatment of maple syrup disease: A study of 36 patients
    • DOI 10.1542/peds.109.6.999
    • DH Morton KA Strauss DL Robinson 2002 Diagnosis and treatment of maple syrup disease: A study of 36 patients Pediatrics 109 999 1008 10.1542/peds.109.6.999 12042535 (Pubitemid 34587423)
    • (2002) Pediatrics , vol.109 , Issue.6 , pp. 999-1008
    • Holmes Morton, D.1    Strauss, K.A.2    Robinson, D.L.3    Puffenberger, E.G.4    Kelley, R.I.5
  • 26
    • 0036213208 scopus 로고    scopus 로고
    • Lipid status and long-chain polyunsaturated fatty acid concentrations in adults and adolescents with phenylketonuria on phenylalanine-restricted diet
    • DOI 10.1023/A:1015142001578
    • K Moseley R Koch AB Moser 2002 Lipid status and long-chain polyunsaturated fatty acid concentrations in adults and adolescents with phenylketonuria on phenylalanine-restricted diet J Inherit Metab Dis 25 1 56 64 10.1023/A:1015142001578 1:CAS:528:DC%2BD38XjvVKlsbw%3D 11999981 (Pubitemid 34303227)
    • (2002) Journal of Inherited Metabolic Disease , vol.25 , Issue.1 , pp. 56-64
    • Moseley, K.1    Koch, R.2    Moser, A.B.3
  • 27
    • 0034884757 scopus 로고    scopus 로고
    • Physiological compartmental analysis of alpha-linolenic acid metabolism in adult humans
    • 1:CAS:528:DC%2BD3MXlvFeksL8%3D 11483627
    • RJ Pawlosky JR Hibbeln JA Novotny N Salem 2001 Physiological compartmental analysis of alpha-linolenic acid metabolism in adult humans J Lipid Res 42 8 1257 1265 1:CAS:528:DC%2BD3MXlvFeksL8%3D 11483627
    • (2001) J Lipid Res , vol.42 , Issue.8 , pp. 1257-1265
    • Pawlosky, R.J.1    Hibbeln, J.R.2    Novotny, J.A.3    Salem, N.4
  • 28
    • 0000758159 scopus 로고
    • Disorders of proline and hydroxyproline metabolism
    • C.R. Scriver A.L. Beaudet W.S. Sly D. Valle (eds). 6 McGraw-Hill New York
    • Phang JM, Scriver CR (1989) Disorders of proline and hydroxyproline metabolism. In: Scriver CR, Beaudet AL, Sly WS, Valle D (eds) The metabolic basis of inherited disease, vol. I, 6th edn. McGraw-Hill, New York, pp 577-597
    • (1989) The Metabolic Basis of Inherited Disease, Vol. i , pp. 577-597
    • Phang, J.M.1    Scriver, C.R.2
  • 29
    • 0031850932 scopus 로고    scopus 로고
    • Long-chain polyunsaturated fatty acids in plasma and erythrocyte membrane lipids of children with phenylketonuria after controlled linoleic acid intake
    • DOI 10.1023/A:1005350523826
    • AP Poge K Baumann E Muller 1998 Long-chain polyunsaturated fatty acids in plasma and erythrocyte membrane lipids of children with phenylketonuria after controlled linoleic acid intake J Inherit Metab Dis 21 4 373 381 10.1023/A:1005350523826 1:CAS:528:DyaK1cXlt1ahtr0%3D 9700594 (Pubitemid 28356419)
    • (1998) Journal of Inherited Metabolic Disease , vol.21 , Issue.4 , pp. 373-381
    • Poge, A.P.1    Baumann, K.2    Muller, E.3    Leichsenring, M.4    Schmidt, H.5    Bremer, H.J.6
  • 30
    • 31344460354 scopus 로고    scopus 로고
    • Assessment of erythrocyte phospholipid fatty acid composition as a biomarker for dietary MUFA, PUFA or saturated fatty acid intake in a controlled cross-over intervention trial
    • DOI 10.1186/1476-511X-4-30
    • SD Poppitt P Kilmartin P Butler 2005 Assessment of erythrocyte phospholipids fatty acid composition as a biomarker for dietary MUFA. PUFA or saturated fatty acid intake in a controlled cross-over intervention trial Lipids Health Dis 4 30 10.1186/1476-511X-4-30 16329761 (Pubitemid 43136443)
    • (2005) Lipids in Health and Disease , vol.4 , pp. 30
    • Poppitt, S.D.1    Kilmartin, P.2    Butler, P.3    Keogh, G.F.4
  • 31
    • 0037321753 scopus 로고    scopus 로고
    • Biosynthesis of docosahexaenoic acid (DHA, 22:6-4, 7,10,13,16,19): Two distinct pathways
    • DOI 10.1016/S0952-3278(02)00268-5
    • X Qiu 2003 Biosynthesis of docosahexaenoic acid (DHA, 22:6-4, 7, 10, 13, 16, 19): two distinct pathways Prostaglandins Leukot Essent Fatty Acids 68 181 186 10.1016/S0952-3278(02)00268-5 1:CAS:528:DC%2BD3sXltlShtA%3D%3D 12538082 (Pubitemid 36216482)
    • (2003) Prostaglandins Leukotrienes and Essential Fatty Acids , vol.68 , Issue.2 , pp. 181-186
    • Qiu, X.1
  • 32
    • 24044498990 scopus 로고    scopus 로고
    • Long-chain n-3 polyunsaturated fatty acids in plasma in British meat-eating, vegetarian, and vegan men
    • 1:CAS:528:DC%2BD2MXosVeisrw%3D 16087975
    • MS Rosell Z Lloyd-Wright PN Appleby, et al. 2005 Long-chain n-3 polyunsaturated fatty acids in plasma in British meat-eating, vegetarian, and vegan men Am J Clin Nutr 82 2 327 334 1:CAS:528:DC%2BD2MXosVeisrw%3D 16087975
    • (2005) Am J Clin Nutr , vol.82 , Issue.2 , pp. 327-334
    • Rosell, M.S.1    Lloyd-Wright, Z.2    Appleby, P.N.3
  • 33
    • 0018188345 scopus 로고
    • Studies of vegans: The fatty acid composition of plasma choline phosphoglycerides, erythrocytes, adipose tissue, and breast milk, and some indicators of susceptibility to ischemic heart disease in vegans and omnivore controls
    • 1:CAS:528:DyaE1cXkslClu7k%3D 645628
    • TA Sanders FR Ellis JW Dickerson 1978 Studies of vegans: the fatty acid composition of plasma choline phosphoglycerides, erythrocytes, adipose tissue, and breast milk, and some indicators of susceptibility to ischemic heart disease in vegans and omnivore controls Am J Clin Nutr 31 5 805 813 1:CAS:528:DyaE1cXkslClu7k%3D 645628
    • (1978) Am J Clin Nutr , vol.31 , Issue.5 , pp. 805-813
    • Sanders, T.A.1    Ellis, F.R.2    Dickerson, J.W.3
  • 34
    • 1942425094 scopus 로고    scopus 로고
    • Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease
    • DOI 10.1016/j.ymgme.2004.01.016, PII S1096719204000496
    • S Schonberger B Shweiger B Schwahn 2004 Dysmyelination in the brain of adolescents and young adults with maple syrup urine disease Mol Genet Metab 82 69 75 10.1016/j.ymgme.2004.01.016 1:CAS:528:DC%2BD2cXjtl2mu7w%3D 15110325 (Pubitemid 38521132)
    • (2004) Molecular Genetics and Metabolism , vol.82 , Issue.1 , pp. 69-75
    • Schonberger, S.1    Schweiger, B.2    Schwahn, B.3    Schwarz, M.4    Wendel, U.5
  • 35
    • 34247610414 scopus 로고    scopus 로고
    • Social outcome in adults with maple syrup urine disease (MSUD)
    • 10.1007/s10545-007-0475-4 1:STN:280:DC%2BD2s7ntFSluw%3D%3D 17310329
    • E Simon M Shwarz U Wendel 2007 Social outcome in adults with maple syrup urine disease (MSUD) J Inherit Metab Dis 30 264 10.1007/s10545-007-0475-4 1:STN:280:DC%2BD2s7ntFSluw%3D%3D 17310329
    • (2007) J Inherit Metab Dis , vol.30 , pp. 264
    • Simon, E.1    Shwarz, M.2    Wendel, U.3
  • 36
    • 0034218476 scopus 로고    scopus 로고
    • Impact of a camp experience on phenylalanine levels, knowledge, attitudes, and health beliefs relevant to nutrition management of phenylketonuria in adolescent girls
    • 10.1016/S0002-8223(00)00232-7 1:STN:280:DC%2BD3czntlagsg%3D%3D 10916518
    • RH Singh JA Kable NV Guerrero KM Sullivan LJ Elsas 2000 Impact of a camp experience on phenylalanine levels, knowledge, attitudes, and health beliefs relevant to nutrition management of phenylketonuria in adolescent girls J Am Diet Assoc 100 797 803 10.1016/S0002-8223(00)00232-7 1:STN:280: DC%2BD3czntlagsg%3D%3D 10916518
    • (2000) J Am Diet Assoc , vol.100 , pp. 797-803
    • Singh, R.H.1    Kable, J.A.2    Guerrero, N.V.3    Sullivan, K.M.4    Elsas, L.J.5
  • 37
    • 33644842334 scopus 로고    scopus 로고
    • Dietary-induced changes in fatty acid composition of human plasma, platelet, and erythrocyte lipids follow a similar time course
    • 1:CAS:528:DC%2BD28XitVOrsbw%3D 16484525
    • CM Skeaff L Hodson JE McKenzie 2006 Dietary-induced changes in fatty acid composition of human plasma, platelet, and erythrocyte lipids follow a similar time course J Nutr 136 565 569 1:CAS:528:DC%2BD28XitVOrsbw%3D 16484525
    • (2006) J Nutr , vol.136 , pp. 565-569
    • Skeaff, C.M.1    Hodson, L.2    McKenzie, J.E.3
  • 40
    • 0041488073 scopus 로고    scopus 로고
    • Branched-chain ketoacyl dehydrogenase deficiency: Maple syrup disease
    • 10.1007/s11940-003-0039-3 12791200
    • KA Strauss DH Morton 2003 Branched-chain ketoacyl dehydrogenase deficiency: maple syrup disease Curr Treat Options Neurol 5 329 341 10.1007/s11940-003-0039-3 12791200
    • (2003) Curr Treat Options Neurol , vol.5 , pp. 329-341
    • Strauss, K.A.1    Morton, D.H.2
  • 41
    • 0034543121 scopus 로고    scopus 로고
    • The essential fatty acid status in phenylketonuria patients under treatment
    • CJAW Van Gool AC van Houwelingen G Hornstra 2002 The essential fatty acid status in phenylketonuria patients under treatment J Nutr Biochem 11 543 547
    • (2002) J Nutr Biochem , vol.11 , pp. 543-547
    • Van Gool Cjaw1    Van Houwelingen, A.C.2    Hornstra, G.3


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.