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苯丙氨酸羟化酶的同工酶

Isozymes of phenylalanine hydroxylase.

作者信息

Barranger J A, Geiger P J, Huzino A, Bessman S P

出版信息

Science. 1972 Feb 25;175(4024):903-5. doi: 10.1126/science.175.4024.903.

DOI:10.1126/science.175.4024.903
PMID:4257648
Abstract

Three isozymes of phenylalanine hydroxylase exist in adult rat liver. They are chromatographically unique. Partial chracterization suggests that they are similar in chemical properties and differ only in charge. Estimation of the Stokes radii indicates that the isozymes have similar molecular weights of about 200,000. Two isozymes exist in human fetal liver. Alterations of the relative amounts of these isozymes may control the phenotype of the disease phenylketonuria.

摘要

成年大鼠肝脏中存在三种苯丙氨酸羟化酶同工酶。它们在色谱上具有独特性。部分特性表明它们在化学性质上相似,仅电荷不同。斯托克斯半径的估计表明这些同工酶的分子量相似,约为200,000。人类胎儿肝脏中存在两种同工酶。这些同工酶相对含量的改变可能控制苯丙酮尿症的疾病表型。

相似文献

1
Isozymes of phenylalanine hydroxylase.苯丙氨酸羟化酶的同工酶
Science. 1972 Feb 25;175(4024):903-5. doi: 10.1126/science.175.4024.903.
2
Phenylalanine hydroxylase activity in human foetal fibroblastic cells in culture.培养的人胎儿成纤维细胞中的苯丙氨酸羟化酶活性
Biochim Biophys Acta. 1972 Mar 30;264(1):205-9. doi: 10.1016/0304-4165(72)90132-8.
3
The isolation and properties of phenylalanine hydroxylase from human liver.人肝脏苯丙氨酸羟化酶的分离及其性质
Biochem J. 1974 Jun;139(3):741-9. doi: 10.1042/bj1390741.
4
In vitro effects of L-and D-parachlorophenylalanine on rat liver phenylalanine hydroxylase.
Biochimie. 1980;62(2-3):185-6. doi: 10.1016/s0300-9084(80)80195-7.
5
Rat liver phenylalanine hydroxylase, an iron enzyme.大鼠肝脏苯丙氨酸羟化酶,一种含铁酶。
J Biol Chem. 1972 Aug 25;247(16):5161-7.
6
Inhibition of phenylalanine hydroxylase by p-chlorophenylalanine; dependence on cofactor structure.对氯苯丙氨酸对苯丙氨酸羟化酶的抑制作用;对辅因子结构的依赖性。
Biochem Biophys Res Commun. 1974 Nov 6;61(1):360-6. doi: 10.1016/0006-291x(74)90575-0.
7
Detection of hepatic phenylalanine 4-hydroxylase in classical phenylketonuria.经典型苯丙酮尿症中肝脏苯丙氨酸4-羟化酶的检测
Proc Natl Acad Sci U S A. 1973 Feb;70(2):552-6. doi: 10.1073/pnas.70.2.552.
8
The effects in vivo of L- and D-para-chlorophenylalanine on the aromatic hydroxylating activity of rat liver.
Can J Biochem. 1972 Feb;50(2):151-3. doi: 10.1139/o72-019.
9
The isolation, properties, and assay of phenylalanine hydroxylase from human and rat liver.从人和大鼠肝脏中分离、鉴定苯丙氨酸羟化酶并进行分析测定。
Biochem Med. 1976 Dec;16(3):284-91. doi: 10.1016/0006-2944(76)90034-x.
10
In vivo inhibition of rat liver phenylalanine hydroxylase by p-chlorophenylalanine and Esculin. Experimental model of phenylketonuria.对氯苯丙氨酸和七叶苷对大鼠肝脏苯丙氨酸羟化酶的体内抑制作用。苯丙酮尿症的实验模型。
Biochem Med. 1975 Jan;12(1):72-8. doi: 10.1016/0006-2944(75)90097-6.

引用本文的文献

1
Gene Therapy for the Treatment of Neurological Disorders: Metabolic Disorders.用于治疗神经疾病的基因疗法:代谢紊乱
Methods Mol Biol. 2016;1382:429-65. doi: 10.1007/978-1-4939-3271-9_30.
2
The isolation and properties of phenylalanine hydroxylase from rat liver.大鼠肝脏苯丙氨酸羟化酶的分离与性质
Biochem J. 1974 Jun;139(3):731-9. doi: 10.1042/bj1390731.
3
The isolation and properties of phenylalanine hydroxylase from human liver.人肝脏苯丙氨酸羟化酶的分离及其性质
Biochem J. 1974 Jun;139(3):741-9. doi: 10.1042/bj1390741.
4
Phenylalaninaemia. Differential diagnosis.苯丙酮尿症。鉴别诊断。
Arch Dis Child. 1974 Nov;49(11):835-43. doi: 10.1136/adc.49.11.835.
5
Regional mapping of the phenylalanine hydroxylase gene and the phenylketonuria locus in the human genome.
Proc Natl Acad Sci U S A. 1985 Sep;82(18):6221-5. doi: 10.1073/pnas.82.18.6221.
6
Hepatic phenylalanine 4-monooxygenase is a phosphoprotein.肝脏苯丙氨酸4-单加氧酶是一种磷蛋白。
Proc Natl Acad Sci U S A. 1976 May;73(5):1591-3. doi: 10.1073/pnas.73.5.1591.
7
Genetics of the mammalian phenylalanine hydroxylase system. II. Immunological and two-dimensional gel electrophoretic studies of phenylalanine hydroxylase in cultured normal and mutant rat hepatoma cells.哺乳动物苯丙氨酸羟化酶系统的遗传学。II. 培养的正常和突变大鼠肝癌细胞中苯丙氨酸羟化酶的免疫学和二维凝胶电泳研究。
Biochem Genet. 1979 Oct;17(9-10):921-46. doi: 10.1007/BF00504313.
8
Phenylketonuria and its variants.苯丙酮尿症及其变异型。
West J Med. 1979 Oct;131(4):324-6.
9
Diseases of phenylalanine metabolism.苯丙氨酸代谢疾病。
West J Med. 1979 Oct;131(4):285-97.
10
Abnormal amino acid metabolism and brain protein synthesis during neural development.神经发育过程中异常的氨基酸代谢和脑蛋白合成。
Neurochem Res. 1978 Aug;3(4):381-99. doi: 10.1007/BF00966321.