HYPERPROLINEMIA TYPE-II - EVIDENCE OF THE EXCRETION OF 3-HYDROXY DELTA-1-PYRROLINE 5-CARBOXYLIC ACID

被引:2
作者
DOOLEY, KC
APPLEGARTH, DA
机构
[1] CHILDRENS HOSP, BIOCHEM DIS LAB, VANCOUVER V5X 1X2, BC, CANADA
[2] UNIV BRITISH COLUMBIA, DEPT PEDIAT, VANCOUVER V6T 1W5, BC, CANADA
关键词
D O I
10.1016/S0009-9120(79)80009-0
中图分类号
R446 [实验室诊断]; R-33 [实验医学、医学实验];
学科分类号
1001 ;
摘要
.DELTA.1-Pyrroline 3-hydroxy 5-carboxylic acid (hydroxy PCA) was identified in the urine of a previously reported patient with hyperprolinemia Type II. This compound was previously called pseudo PCA.
引用
收藏
页码:62 / 65
页数:4
相关论文
共 7 条
[1]  
ADAMS E, 1960, J BIOL CHEM, V235, P3492
[2]   HYPERPROLINEMIA TYPE-II - IDENTIFICATION OF GLYCINE CONJUGATE OF PYRROLE-2-CARBOXYLIC ACID IN URINE [J].
APPLEGARTH, DA ;
GOODMAN, SI ;
IRVINE, DG ;
JELLUM, E .
CLINICAL BIOCHEMISTRY, 1977, 10 (01) :20-23
[3]   HYPERPROLINEMIA TYPE-II [J].
APPLEGARTH, DA ;
INGRAM, P ;
HINGSTON, J ;
HARDWICK, DF .
CLINICAL BIOCHEMISTRY, 1974, 7 (01) :14-28
[4]   DEFECTIVE HYDROXYPROLINE METABOLISM IN TYPE-II HYPERPROLINEMIA [J].
GOODMAN, SI ;
MACE, JW ;
MILES, BS ;
TENG, CC ;
BROWN, SB .
BIOCHEMICAL MEDICINE, 1974, 10 (04) :329-336
[5]   DELTA-1-PYRROLINE-5-CARBOXYLATE AND DELTA-1-PYRROLINE-3-HYDROXY-5-CARBOXYLATE - CHROMATOGRAPHY ON AMINO-ACID ANALYZER [J].
HEACOCK, AM ;
WILLIAMS, IH ;
FRANK, LH ;
ADAMS, E .
ANALYTICAL BIOCHEMISTRY, 1975, 64 (02) :593-600
[6]   TYPE-2 HYPERPROLINEMIA DELTA 1-PYRROLINE-5-CARBOXYLIC ACID DEHYDROGENASE-DEFICIENCY IN CULTURED SKIN FIBROBLASTS AND CIRCULATING LYMPHOCYTES [J].
VALLE, D ;
GOODMAN, SI ;
APPLEGARTH, DA ;
SHIH, VE ;
PHANG, JM .
JOURNAL OF CLINICAL INVESTIGATION, 1976, 58 (03) :598-603
[7]   TYPE 2 HYPERPROLINEMIA - ABSENCE OF DELTA-1-PYRROLINE-5-CARBOXYLIC ACID DEHYDROGENASE-ACTIVITY [J].
VALLE, DL ;
PHANG, JM ;
GOODMAN, SI .
SCIENCE, 1974, 185 (4156) :1053-1054