3-HYDROXYPROPIONATE. SIGNIFICANCE OF BETA -OXIDATION OF PROPIONATE IN PATIENTS WITH PROPIONIC ACIDEMI AND METHYL MALONIC ACIDEMIAANDO T; RASMUSSEN K; NYHAN WL et al.1972; PROC. NATION. ACAD. SCI. U.S.A.; U.S.A.; DA. 1972; VOL. 69; NO 10; PP. 2807-2811; BIBL. 29 REF.Serial Issue

HYPERPIPECOLIC ACIDEMIA: CLINICAL AND BIOCHEMICAL OBSERVATIONS IN TWO MALE SIBLINGSBURTON BK; REED SP; REMY WT et al.1981; J. PEDIATR.; ISSN 0022-3476; USA; DA. 1981; VOL. 99; NO 5; PP. 729-734; BIBL. 14 REF.Article

POSSIBLE HAZARDS OF NEOMYCIN TREATMENT IN METHYLMALONIC ACIDEMIA = DANGERS POSSIBLES DU TRAITEMENT PAR LA NEOMYCINE DANS L'ACIDEMIE METHYLMALONIQUEDURAN M; BAKKER HD; WADMAN SK et al.1974; PEDIATRICS; U.S.A.; DA. 1974; VOL. 53; NO 5 PART. 1; PP. 765-766; BIBL. 4REF.Article

ISOVALERIC ACIDEMIA. IDENTIFICATION OF ISOVALERATE, ISOVALERYLGLYCINE, AND 3-HYDROXYISOVALERATE IN URINE OF A PATIENT PREVIOUSLY REPORTED AS HAVING BUTYRIC AND HEXANOIC ACIDEMIA = ACIDEMIE ISOVALERIQUE: IDENTIFICATION DE L'ISOVALERATE, DE L'ISOVALERYLGLYCINE ET DU 3-HYDROXYISOVALERATE DANS LES URINES D'UN MALADE PRESENTE ANTERIEUREMENT COMME SOUFFRANT D'UNE ACIDEMIE BUTYRIQUE ET HEXANOIQUEANDO T; NYHAN WL; BACHMANN C et al.1973; J. PEDIATR.; U.S.A.; DA. 1973; VOL. 82; NO 2; PP. 243-248; BIBL. 13REF.Serial Issue

THE USE OF NEOMYCIN IN THE TREATMENT OF METHYLMALONIC ACIDURIA = L'EMPLOI DE LA NEOMYCINE DANS LE TRAITEMENT DE L'ACIDURIE METHYLMALONIQUESNYDERMAN SE; SANSARICQ C; NORTON P et al.1972; PEDIATRICS; U.S.A.; DA. 1972; VOL. 50; NO 6; PP. 925-927; BIBL. 7 REF.Serial Issue

EXCRETION OF PROPIONYLGLYCINE IN PROPIONIC ACIDAEMIA = L'EXCRETION DE PROPIONYLGLYCINE DANS L'ACIDEMIE PROPIONIQUERASMUSSEN K; ANDO T; NYHAN WL et al.1972; CLIN. SCI.; G.B.; DA. 1972; VOL. 42; NO 6; PP. 665-671; BIBL. 1P.Serial Issue

NEW METABOLITES IN ISOVALERIC ACIDEMIATRUSCOTT RJW; MALEGAN D; MCCAIRNS E et al.1981; CLIN. CHIM. ACTA; ISSN 0009-8981; NLD; DA. 1981; VOL. 110; NO 2-3; PP. 187-203; BIBL. 31 REF.Article

THE INHIBITION BY METHYLMALONIC ACID OF MALATE TRANSPORT BY THE DICARBOXYLATE CARRIER IN RAT LIVER MITOCHONDRIA. A POSSIBLE EXPLANATION FOR HYPOGLYCEMIA IN METHYLMALONIC ACIDURIA = INHIBITION PAR L'ACIDE METHYLMALONIQUE DU TRANSPORT DE L'ACIDE MALIQUE PAR L'ACIDE DICARBOXYLIQUE VECTEUR DANS LES MITOCHONDRIES DU FOIE DU RAT. EXPLICATION POSSIBLE DE L'HYPOGLYCEMIE DE L'ACIDURIE METHYLMALONIQUEHALPERIN ML; SCHILLER CM; FRITZ IB et al.1971; J. CLIN. INVEST.; U.S.A.; DA. 1971; VOL. 50; NO 11; PP. 2276-2282; BIBL. 24REF.Serial Issue

GLUTARIC ACIDURIA; A NEW DISORDER OF AMINO ACID METABOLISM = ACIDURIE GLUTARIQUE: UN NOUVEAU TROUBLE DU METABOLISME DES ACIDES AMINESGOODMAN SI; MARKEY SP; MOE PG et al.1975; BIOCHEM. MED.; U.S.A.; DA. 1975; VOL. 12; NO 1; PP. 12-21; BIBL. 1P.Article

PROPIONYL-COA CARBOXYLASE DEFICIENCY: CASE REPORT, EFFECT OF LOW-PROTEIN DIET AND IDENTIFICATION OF 3-OXO-2-METHYLVALERIC ACID 3-HYDROXY-2-METHYLVALERIC ACID, AND MALEIC ACID IN URINEBERGSTROM T; GRETER J; LEVIN AH et al.1981; SCAND. J. CLIN. LAB. INVEST.; ISSN 0036-5513; NOR; DA. 1981; VOL. 41; NO 2; PP. 117-126; BIBL. 37 REF.Article

METHYLMALONICACIDEMIA: BIOCHEMICAL HETEROGENEITY IN DEFECTS OF 5'-DEOXYADENOSYLCOBALAMIN SYNTHESIS.MAHONEY MJ; HART AC; STEEN VD et al.1975; PROC. NATION. ACAD. SCI.; U.S.A.; DA. 1975; VOL. 72; NO 7; PP. 2799-2903; BIBL. 30 REF.Article

(¹³C) VALINE METABOLISM IN METHYLMALONICACIDEMIA USING NUCLEAR MAGNETIC RESONANCE: PROPIONATE AS AN OBLIGATE INTERMEDIATE.TANAKA K; ARMITAGE IM; RAMSDELL HS et al.1975; PROC. NATION. ACAD. SCI., U.S.A.; U.S.A.; DA. 1975; VOL. 72; NO 9; PP. 3692-3696; BIBL. 25 REF.Article

PYRUVATE CARBOXYLASE IN HUMAN LIVER. APPARENT LOSS OF A COMPONENT OF CATALYTIC ACTIVITY IN A FORM OF LACTIC ACIDOSIS WITH HYPOGLYCEMIA = PYRUVATE CARBOXYLASE DU FOIE HUMAIN. PERTE APPARENTE D'UNE COMPOSANTE DE L'ACTIVITE CATALITIQUE SOUS FORME D'ACIDOSE LACTIQUE AVEC HYPOGLYCEMIEDELVIN E; SCRIVER CR; NEAL JL et al.1974; BIOCHEM. MED.; U.S.A.; DA. 1974; VOL. 10; NO 2; PP. 97-106; BIBL. 21REF.Article

IN VITRO RESPONSIVE METHYLMALONIC ACIDEMIA: A NEW VARIANT = ACIDEMIE METHYLMALONIQUE CURABLE IN VITRO: UNE NOUVELLE VARIANTEKAYE CI; MORROW G III; NADLER HL et al.1974; J. PEDIATR.; U.S.A.; DA. 1974; VOL. 85; NO 1; PP. 55-59; BIBL. 21REF.Article

PHENYLKETONURIA AND OTHER PHENYLALANINAEMIAS = PHENYLCETONURIE ET AUTRES PHENYLALANINEMIESBLASKOVICS ME.1974; CLIN. ENDOCRINOL. METABOL.; G.B.; DA. 1974; VOL. 3; NO 1; PP. 87-105; BIBL. 1P.Article

EFFECTS OF UREA HYDROLYSIS ON TISSUE METABOLITE CONCENTRATIONS IN RATS = EFFETS DE L'HYDROLYSE DE L'UREE SUR LES CONCENTRATIONS DE METABOLITE TISSULAIRE CHEZ LE RATPRIOR RL; VISEK WJ.1972; AMER. J. PHYSIOL.; U.S.A.; DA. 1972; VOL. 223; NO 5; PP. 1143-1149; BIBL. 38 REF.Serial Issue

ACIDEMIE METHYL-MALONIQUE AVEC NEPHROPATHIE HYPERURICEMIQUEBROYER M; GUESRY P; BURGESS EA et al.1974; ARCH. FR. PEDIATR.; FR.; DA. 1974; VOL. 31; NO 6; PP. 543-552; ABS. ANGL.; BIBL. 11REF.Article

OCCURRENCE OF HYPERAMMONEMIA IN THE COURSE OF 17 CASES OF METHYLMALONIC ACIDEMIACATHELINEAU L; BRIAND P; OGIER H et al.1981; J. PEDIATR.; ISSN 0022-3476; USA; DA. 1981; VOL. 99; NO 2; PP. 279-280; BIBL. 10 REF.Article

3-HYDROXYISOHEPTANOIC ACID. A NEW METABOLITE IN ISOVALERIC ACIDEMIALEHNERT W.1981; CLIN. CHIM. ACTA; ISSN 0009-8981; NLD; DA. 1981; VOL. 113; NO 1; PP. 101-103; BIBL. 7 REF.Article

INCREASED PYROGLUTAMIC ACID LEVELS IN PATIENTS ON ARTIFICIAL DIETS. = AUGMENTATION DES TAUX D'ACIDE PYROGLUTAMIQUE CHEZ LES MALADES A DES REGIMES ARTIFICIELSOBERHOLZER VG; WOOD CBS; PALMER T et al.1975; CLIN. CHIM. ACTA; PAYS-BAS; DA. 1975; VOL. 62; NO 2; PP. 299-304; BIBL. 15 REF.Article

POSSIBILITY OF INBORN DEFECT IN ISOVALERICACIDAEMIA INVOLVING ALTERED ENZYME SPECIFICITY RATHER THAN TOTAL INACTIVITY = POSSIBILITE D'UN DEFAUT CONGENITAL DANS L'ISOVALERICACIDEMIE IMPLIQUANT UNE MODIFICATION DE LA SPECIFICITE ENZYMATIQUE PLUTOT QU'UNE INACTIVITE TOTALEENGEL PC.1974; NATURE; G.B.; DA. 1974; VOL. 248; NO 5444; PP. 140-142; BIBL. 21REF.Article

A METHOD FOR THE SEPARATE DETERMINATION OF ISOVALERATE AND ALPHA -METHYLBUTYRATE BY USE OF GLC-MASS SPECTROMETER = UNE METHODE POUR LE DOSAGE SEPARE DE L'ACIDE ISOVALERIQUE ET DE L'ACIDE ALPHA -METHYLBUTYRIQUE A L'AIDE DU SPECTROMETRE DE MASSE GLCTANAKA K; YU GM.1973; CLIN. CHIM. ACTA,; PAYS-BAS; DA. 1973; VOL. 43; NO 1; PP. 151-154; BIBL. 6REF.Serial Issue

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