Endogenous synthesis of galactose in normal men and patients with hereditary galactosaemia
Despite restricted ingestion of lactose, patients with galactose-1–phosphate uridyltransferase deficiency have raised concentrations of galactose metabolites in blood and urine. Endogenous production of galactose may underline this phenomenon. Using isotopically labelled galactose in a continuous in...
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| Veröffentlicht in: | The Lancet (British edition) 1995-10, Vol.346 (8982), p.1073-1074 |
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| creator | Berry, G.T. Nissim, I. Lin, Z. Mazur, A.T. Gibson, J.B. Segal, S. |
| description | Despite restricted ingestion of lactose, patients with galactose-1–phosphate uridyltransferase deficiency have raised concentrations of galactose metabolites in blood and urine. Endogenous production of galactose may underline this phenomenon. Using isotopically labelled galactose in a continuous intravenous infusion, we employed the steady-state flux method to calculate endogenous galactose production rate in three normal men and three patients with classic galactosaemia. We found that galactosaemic patients and normal subjects synthesise gram quantities of galactose per day. The rate of synthesis ranged from 0·53–1·05 mg/kg per h. Endogenous production of galactose may be an important factor in the pathogenesis of the complications of the brain and ovary, and could explain the persistent elevation of galactose metabolites in patients despite dietary restriction of galactose. |
| doi_str_mv | 10.1016/S0140-6736(95)91745-4 |
| format | Article |
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Endogenous production of galactose may underline this phenomenon. Using isotopically labelled galactose in a continuous intravenous infusion, we employed the steady-state flux method to calculate endogenous galactose production rate in three normal men and three patients with classic galactosaemia. We found that galactosaemic patients and normal subjects synthesise gram quantities of galactose per day. The rate of synthesis ranged from 0·53–1·05 mg/kg per h. Endogenous production of galactose may be an important factor in the pathogenesis of the complications of the brain and ovary, and could explain the persistent elevation of galactose metabolites in patients despite dietary restriction of galactose.</description><identifier>ISSN: 0140-6736</identifier><identifier>EISSN: 1474-547X</identifier><identifier>DOI: 10.1016/S0140-6736(95)91745-4</identifier><identifier>PMID: 7564790</identifier><identifier>CODEN: LANCAO</identifier><language>eng</language><publisher>London: Elsevier Ltd</publisher><subject>Adult ; Biological and medical sciences ; Blood ; Brain ; Carbohydrates (enzymatic deficiencies). Glycogenosis ; Dietary restrictions ; Errors of metabolism ; Female ; Galactose ; Galactose - biosynthesis ; Galactosemia ; Galactosemias - diet therapy ; Galactosemias - genetics ; Galactosemias - metabolism ; Genetics ; Humans ; Ingestion ; Intravenous administration ; Intravenous infusion ; Lactose ; Male ; Medical research ; Medical sciences ; Men ; Metabolic diseases ; Metabolism ; Metabolites ; Pathogenesis ; Synthesis ; Urine</subject><ispartof>The Lancet (British edition), 1995-10, Vol.346 (8982), p.1073-1074</ispartof><rights>1995</rights><rights>1995 INIST-CNRS</rights><rights>Copyright Lancet Ltd. 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Endogenous production of galactose may underline this phenomenon. Using isotopically labelled galactose in a continuous intravenous infusion, we employed the steady-state flux method to calculate endogenous galactose production rate in three normal men and three patients with classic galactosaemia. We found that galactosaemic patients and normal subjects synthesise gram quantities of galactose per day. The rate of synthesis ranged from 0·53–1·05 mg/kg per h. Endogenous production of galactose may be an important factor in the pathogenesis of the complications of the brain and ovary, and could explain the persistent elevation of galactose metabolites in patients despite dietary restriction of galactose.</description><subject>Adult</subject><subject>Biological and medical sciences</subject><subject>Blood</subject><subject>Brain</subject><subject>Carbohydrates (enzymatic deficiencies). Glycogenosis</subject><subject>Dietary restrictions</subject><subject>Errors of metabolism</subject><subject>Female</subject><subject>Galactose</subject><subject>Galactose - biosynthesis</subject><subject>Galactosemia</subject><subject>Galactosemias - diet therapy</subject><subject>Galactosemias - genetics</subject><subject>Galactosemias - metabolism</subject><subject>Genetics</subject><subject>Humans</subject><subject>Ingestion</subject><subject>Intravenous administration</subject><subject>Intravenous infusion</subject><subject>Lactose</subject><subject>Male</subject><subject>Medical research</subject><subject>Medical sciences</subject><subject>Men</subject><subject>Metabolic diseases</subject><subject>Metabolism</subject><subject>Metabolites</subject><subject>Pathogenesis</subject><subject>Synthesis</subject><subject>Urine</subject><issn>0140-6736</issn><issn>1474-547X</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>1995</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>8G5</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>GUQSH</sourceid><sourceid>M2O</sourceid><recordid>eNqFkU9rFTEUxYNY6rP6EQpBRexi9GYmfyYrkVKtUHChgrgJmeROX8pM8kzmKf32pn2PtyiIq7u4v3M59xxCThm8ZcDku6_AODRSdfKNFmeaKS4a_oisGFe8EVz9eExWB-QJeVrKDQBwCeKYHCshudKwIj8vok_XGNO20HIblzWWUGga6bWdrFtSQRoijSnPdqIzRmqjpxu7BIxLoX_CsqZrzOjDYvPtQWRxDvYZORrtVPD5fp6Q7x8vvp1fNldfPn0-_3DVOC563nAugLfeO92JVrWiU-PggGsEpocBqnkmFWMCnZTQAhsYCD160L73PWddd0Je7-5ucvq1xbKYORSH02Qj1reMqs9K0ekKvnwA3qRtjtWbaVvGtRC8U5V68S-K6V5Xh72skNhBLqdSMo5mk8NcIzAMzF095r4ec5e90cLc12N41Z3uj2-HGf1Bte-j7l_t97Y4O43ZRhfKAeuk7ntoK_Z-h2HN9XfAbIqrjbjaQ0a3GJ_Cf4z8BeAXqcI</recordid><startdate>19951021</startdate><enddate>19951021</enddate><creator>Berry, G.T.</creator><creator>Nissim, I.</creator><creator>Lin, Z.</creator><creator>Mazur, A.T.</creator><creator>Gibson, J.B.</creator><creator>Segal, S.</creator><general>Elsevier Ltd</general><general>Lancet</general><general>Elsevier Limited</general><scope>IQODW</scope><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>0TT</scope><scope>0TZ</scope><scope>0U~</scope><scope>3V.</scope><scope>7QL</scope><scope>7QP</scope><scope>7RV</scope><scope>7TK</scope><scope>7U7</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88A</scope><scope>88C</scope><scope>88E</scope><scope>88G</scope><scope>88I</scope><scope>8AF</scope><scope>8AO</scope><scope>8C1</scope><scope>8C2</scope><scope>8FE</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>8G5</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AN0</scope><scope>ASE</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BEC</scope><scope>BENPR</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FPQ</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>GUQSH</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K6X</scope><scope>K9-</scope><scope>K9.</scope><scope>KB0</scope><scope>KB~</scope><scope>LK8</scope><scope>M0R</scope><scope>M0S</scope><scope>M0T</scope><scope>M1P</scope><scope>M2M</scope><scope>M2O</scope><scope>M2P</scope><scope>M7N</scope><scope>M7P</scope><scope>MBDVC</scope><scope>NAPCQ</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>S0X</scope><scope>7X8</scope></search><sort><creationdate>19951021</creationdate><title>Endogenous synthesis of galactose in normal men and patients with hereditary galactosaemia</title><author>Berry, G.T. ; Nissim, I. ; Lin, Z. ; Mazur, A.T. ; Gibson, J.B. ; Segal, S.</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c4584-445042ddc935272537fbc049e019bb0736167115ec660201b1059fd09d8d84133</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>1995</creationdate><topic>Adult</topic><topic>Biological and medical sciences</topic><topic>Blood</topic><topic>Brain</topic><topic>Carbohydrates (enzymatic deficiencies). 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| subjects | Adult Biological and medical sciences Blood Brain Carbohydrates (enzymatic deficiencies). Glycogenosis Dietary restrictions Errors of metabolism Female Galactose Galactose - biosynthesis Galactosemia Galactosemias - diet therapy Galactosemias - genetics Galactosemias - metabolism Genetics Humans Ingestion Intravenous administration Intravenous infusion Lactose Male Medical research Medical sciences Men Metabolic diseases Metabolism Metabolites Pathogenesis Synthesis Urine |
| title | Endogenous synthesis of galactose in normal men and patients with hereditary galactosaemia |
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