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Volumn 21, Issue SUPPL. 1, 1998, Pages 40-58

The biochemical and molecular spectrum of ornithine transcarbamylase deficiency

Author keywords

[No Author keywords available]

Indexed keywords

AMINO ACID; DINUCLEOTIDE; ORNITHINE; ORNITHINE CARBAMOYLTRANSFERASE; SIGNAL PEPTIDE;

EID: 0031901847     PISSN: 01418955     EISSN: None     Source Type: Journal    
DOI: 10.1023/a:1005353407220     Document Type: Article
Times cited : (95)

References (8)
  • 2
    • 0030967422 scopus 로고    scopus 로고
    • Substrate induced conformational change in a trimeric ornithine transcarbamoylase
    • Ha, Y, McCann MT, Tuchman M, Allewell NM (1997) Substrate induced conformational change in a trimeric ornithine transcarbamoylase. Proc Natl Acad Sci USA 94: 9550-9555.
    • (1997) Proc Natl Acad Sci USA , vol.94 , pp. 9550-9555
    • Ha, Y.1    McCann, M.T.2    Tuchman, M.3    Allewell, N.M.4
  • 3
    • 0020411609 scopus 로고
    • Crystal and molelcular structures of native and CTP ligated aspartate carbamoyltransferase from Esherichia coli
    • Honzatko RB, Crawford JL, Monaco HL, et al (1982) Crystal and molelcular structures of native and CTP ligated aspartate carbamoyltransferase from Esherichia coli. J Mol Biol 160: 219-263.
    • (1982) J Mol Biol , vol.160 , pp. 219-263
    • Honzatko, R.B.1    Crawford, J.L.2    Monaco, H.L.3
  • 4
    • 0023756350 scopus 로고
    • Characterization of point mutations in the same arginine codon in three unrelated patients with ornithine transcarbamylase deficiency
    • Maddalena A, Edward SJ, O'Brien WE, Nussbaum RL (1988) Characterization of point mutations in the same arginine codon in three unrelated patients with ornithine transcarbamylase deficiency. J Clin Invest 82: 1353-1358.
    • (1988) J Clin Invest , vol.82 , pp. 1353-1358
    • Maddalena, A.1    Edward, S.J.2    O'Brien, W.E.3    Nussbaum, R.L.4
  • 5
    • 0031043291 scopus 로고    scopus 로고
    • Expression, purification and kinetic characterization of wild type human ornithine transcarbamylase and a recurrent mutant that produces 'late onset' hyperammonemia
    • Morizono H, Tuchman M, Rajagopal BS, et al (1997a) Expression, purification and kinetic characterization of wild type human ornithine transcarbamylase and a recurrent mutant that produces 'late onset' hyperammonemia. Biochem J 322: 625-631.
    • (1997) Biochem J , vol.322 , pp. 625-631
    • Morizono, H.1    Tuchman, M.2    Rajagopal, B.S.3
  • 6
    • 0030983371 scopus 로고    scopus 로고
    • Late onset ornithine transcarbamylase deficiency: Function of three purified recombinant mutant enzymes
    • Morizono H, Listrom CD, Rajagopal BS, et al (1997b) Late onset ornithine transcarbamylase deficiency: function of three purified recombinant mutant enzymes. Hum Mol Genet 6: 963-968.
    • (1997) Hum Mol Genet , vol.6 , pp. 963-968
    • Morizono, H.1    Listrom, C.D.2    Rajagopal, B.S.3
  • 7
    • 0030040201 scopus 로고    scopus 로고
    • Patient selection may affect gene therapy success. Dominant negative effects observed for ornithine transcarbamylase in mouse and human hepatocytes
    • Morsy MA, Zhao JZ, Ngo TT, et al (1996) Patient selection may affect gene therapy success. Dominant negative effects observed for ornithine transcarbamylase in mouse and human hepatocytes. J Clin Invest 97: 826-832.
    • (1996) J Clin Invest , vol.97 , pp. 826-832
    • Morsy, M.A.1    Zhao, J.Z.2    Ngo, T.T.3
  • 8
    • 0025008168 scopus 로고
    • Sequence logos: A new way to display consensus sequences
    • Schneider TD, Stephens RM (1990) Sequence logos: a new way to display consensus sequences. Nucleic Acids Res 18: 6097-6100.
    • (1990) Nucleic Acids Res , vol.18 , pp. 6097-6100
    • Schneider, T.D.1    Stephens, R.M.2


* 이 정보는 Elsevier사의 SCOPUS DB에서 KISTI가 분석하여 추출한 것입니다.