Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase
Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid...
Ausführliche Beschreibung
Gespeichert in:
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E-Artikel |
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| Sprache: |
Englisch |
| Erschienen: |
1978 |
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| Reproduktion: |
Elsevier Journal Backfiles on ScienceDirect 1907 - 2002 |
|---|---|
| Übergeordnetes Werk: |
in: Clinica Chimica Acta - Amsterdam : Elsevier, 90(1978), 2, Seite 195-200 |
| Übergeordnetes Werk: |
volume:90 ; year:1978 ; number:2 ; pages:195-200 |
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| 245 | 1 | 0 | |a Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase |
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| 520 | |a Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. | ||
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(DE-627)NLEJ186306881 (DE-599)GBVNLZ186306881 DE-627 ger DE-627 rakwb eng Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase 1978 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. Elsevier Journal Backfiles on ScienceDirect 1907 - 2002 Niederwieser, A. oth Wadman, S.K. oth Danks, D.M. oth in Clinica Chimica Acta Amsterdam : Elsevier 90(1978), 2, Seite 195-200 (DE-627)NLEJ184917921 (DE-600)1499920-1 0009-8981 nnns volume:90 year:1978 number:2 pages:195-200 http://linkinghub.elsevier.com/retrieve/pii/0009-8981(78)90522-3 GBV_USEFLAG_H ZDB-1-SDJ GBV_NL_ARTICLE AR 90 1978 2 195-200 |
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(DE-627)NLEJ186306881 (DE-599)GBVNLZ186306881 DE-627 ger DE-627 rakwb eng Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase 1978 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. Elsevier Journal Backfiles on ScienceDirect 1907 - 2002 Niederwieser, A. oth Wadman, S.K. oth Danks, D.M. oth in Clinica Chimica Acta Amsterdam : Elsevier 90(1978), 2, Seite 195-200 (DE-627)NLEJ184917921 (DE-600)1499920-1 0009-8981 nnns volume:90 year:1978 number:2 pages:195-200 http://linkinghub.elsevier.com/retrieve/pii/0009-8981(78)90522-3 GBV_USEFLAG_H ZDB-1-SDJ GBV_NL_ARTICLE AR 90 1978 2 195-200 |
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(DE-627)NLEJ186306881 (DE-599)GBVNLZ186306881 DE-627 ger DE-627 rakwb eng Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase 1978 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. Elsevier Journal Backfiles on ScienceDirect 1907 - 2002 Niederwieser, A. oth Wadman, S.K. oth Danks, D.M. oth in Clinica Chimica Acta Amsterdam : Elsevier 90(1978), 2, Seite 195-200 (DE-627)NLEJ184917921 (DE-600)1499920-1 0009-8981 nnns volume:90 year:1978 number:2 pages:195-200 http://linkinghub.elsevier.com/retrieve/pii/0009-8981(78)90522-3 GBV_USEFLAG_H ZDB-1-SDJ GBV_NL_ARTICLE AR 90 1978 2 195-200 |
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(DE-627)NLEJ186306881 (DE-599)GBVNLZ186306881 DE-627 ger DE-627 rakwb eng Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase 1978 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. Elsevier Journal Backfiles on ScienceDirect 1907 - 2002 Niederwieser, A. oth Wadman, S.K. oth Danks, D.M. oth in Clinica Chimica Acta Amsterdam : Elsevier 90(1978), 2, Seite 195-200 (DE-627)NLEJ184917921 (DE-600)1499920-1 0009-8981 nnns volume:90 year:1978 number:2 pages:195-200 http://linkinghub.elsevier.com/retrieve/pii/0009-8981(78)90522-3 GBV_USEFLAG_H ZDB-1-SDJ GBV_NL_ARTICLE AR 90 1978 2 195-200 |
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(DE-627)NLEJ186306881 (DE-599)GBVNLZ186306881 DE-627 ger DE-627 rakwb eng Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase 1978 nicht spezifiziert zzz rdacontent nicht spezifiziert z rdamedia nicht spezifiziert zu rdacarrier Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. Elsevier Journal Backfiles on ScienceDirect 1907 - 2002 Niederwieser, A. oth Wadman, S.K. oth Danks, D.M. oth in Clinica Chimica Acta Amsterdam : Elsevier 90(1978), 2, Seite 195-200 (DE-627)NLEJ184917921 (DE-600)1499920-1 0009-8981 nnns volume:90 year:1978 number:2 pages:195-200 http://linkinghub.elsevier.com/retrieve/pii/0009-8981(78)90522-3 GBV_USEFLAG_H ZDB-1-SDJ GBV_NL_ARTICLE AR 90 1978 2 195-200 |
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Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase |
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excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase |
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Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase |
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Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. |
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Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. |
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Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid. |
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<?xml version="1.0" encoding="UTF-8"?><collection xmlns="http://www.loc.gov/MARC21/slim"><record><leader>01000caa a22002652 4500</leader><controlfield tag="001">NLEJ186306881</controlfield><controlfield tag="003">DE-627</controlfield><controlfield tag="005">20210707042424.0</controlfield><controlfield tag="007">cr uuu---uuuuu</controlfield><controlfield tag="008">070506s1978 xx |||||o 00| ||eng c</controlfield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-627)NLEJ186306881</subfield></datafield><datafield tag="035" ind1=" " ind2=" "><subfield code="a">(DE-599)GBVNLZ186306881</subfield></datafield><datafield tag="040" ind1=" " ind2=" "><subfield code="a">DE-627</subfield><subfield code="b">ger</subfield><subfield code="c">DE-627</subfield><subfield code="e">rakwb</subfield></datafield><datafield tag="041" ind1=" " ind2=" "><subfield code="a">eng</subfield></datafield><datafield tag="245" ind1="1" ind2="0"><subfield code="a">Excretion of cis- and trans-4-hydroxycyclohexylacetic acid in addition to hawkinsin in a family with a postulated defect of 4-hydroxyphenylpyruvate dioxygenase</subfield></datafield><datafield tag="264" ind1=" " ind2="1"><subfield code="c">1978</subfield></datafield><datafield tag="336" ind1=" " ind2=" "><subfield code="a">nicht spezifiziert</subfield><subfield code="b">zzz</subfield><subfield code="2">rdacontent</subfield></datafield><datafield tag="337" ind1=" " ind2=" "><subfield code="a">nicht spezifiziert</subfield><subfield code="b">z</subfield><subfield code="2">rdamedia</subfield></datafield><datafield tag="338" ind1=" " ind2=" "><subfield code="a">nicht spezifiziert</subfield><subfield code="b">zu</subfield><subfield code="2">rdacarrier</subfield></datafield><datafield tag="520" ind1=" " ind2=" "><subfield code="a">Cis- and trans-4-hydroxycyclohexylacetic acid (4HCHAA) were identified in the urine of a child (suffering from transient tyrosinemia within the first year of life) and her mother, in addition to the recently described new sulfur amino acid (2-cystein-S-yl-1,4-dihydroxycyclohex-5-en-1-yl)acetic acid (hawkinsin, Haw). Identification was possible by gas chromatographic-mass spectrometric analysis of the methyl ester/trimethylsilyl ether and per-trimethylsilyl derivatives, and cochromatography with synthetic reference compounds. Oral loading tests with 3,5-bis-deutero-l-tyrosine have shown, using selected ion monitoring, that 4HCHAA and Haw are tyrosine metabolites. It is postulated that a defective 4-hydroxyphenylpyruvate dioxygenase (EC 1.13.11.27) exists and that Haw and 4HCHAA are derived from (1,2-epoxy-4-hydroxycyclohexa-3,5-dien-1-yl)acetic acid tautomerized to (1,2-epoxycyclohex-5-en-4-on-1-yl)acetic acid.</subfield></datafield><datafield tag="533" ind1=" " ind2=" "><subfield code="f">Elsevier Journal Backfiles on ScienceDirect 1907 - 2002</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Niederwieser, A.</subfield><subfield code="4">oth</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Wadman, S.K.</subfield><subfield code="4">oth</subfield></datafield><datafield tag="700" ind1="1" ind2=" "><subfield code="a">Danks, D.M.</subfield><subfield code="4">oth</subfield></datafield><datafield tag="773" ind1="0" ind2="8"><subfield code="i">in</subfield><subfield code="t">Clinica Chimica Acta</subfield><subfield code="d">Amsterdam : Elsevier</subfield><subfield code="g">90(1978), 2, Seite 195-200</subfield><subfield code="w">(DE-627)NLEJ184917921</subfield><subfield code="w">(DE-600)1499920-1</subfield><subfield code="x">0009-8981</subfield><subfield code="7">nnns</subfield></datafield><datafield tag="773" ind1="1" ind2="8"><subfield code="g">volume:90</subfield><subfield code="g">year:1978</subfield><subfield code="g">number:2</subfield><subfield code="g">pages:195-200</subfield></datafield><datafield tag="856" ind1="4" ind2="0"><subfield code="u">http://linkinghub.elsevier.com/retrieve/pii/0009-8981(78)90522-3</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_USEFLAG_H</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">ZDB-1-SDJ</subfield></datafield><datafield tag="912" ind1=" " ind2=" "><subfield code="a">GBV_NL_ARTICLE</subfield></datafield><datafield tag="951" ind1=" " ind2=" "><subfield code="a">AR</subfield></datafield><datafield tag="952" ind1=" " ind2=" "><subfield code="d">90</subfield><subfield code="j">1978</subfield><subfield code="e">2</subfield><subfield code="h">195-200</subfield></datafield></record></collection>
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