bh4

Gene mutation analysis was performed for patients with BH4 deficiency and their parents .

对BH4缺乏症者及父母进行基因突变检测。

Results In a single BH4 loading test , no classic PKU patients demonstrated decreases in serum phenylalanine levels .

结果在单剂量BH4口服负荷试验中，典型PKU患者的血苯丙氨酸水平没有降低。

Study on the Secondary Metabolites of Sponge-Associated Fungus , Aspergillus sp BH4

海绵来源真菌AspergillusspBH4次级代谢产物的初步研究

BH4 deficient patients were treated with BH4 and neurotransmitter precursors after diagnosis . Blood phenylalanine levels , clinical symptoms and mental development were followed up .

BH4缺乏者一经诊断后立即接受BH4及神经递质前质治疗，追踪随访患者的血Phe浓度、临床症状及智能发育。

Combined loading tests with phenylalanine and oral BH4 were done in 4 of 11 patients and their phenylalanine levels were decreased to normal 4-6h after BH4 administration .

11例中4例作了苯丙氨酸及BH4联合负荷试验，在口服四氢生物蝶呤后4-6小时，他们的血苯丙氨酸水平降至正常。

All cases were diagnosed as BH4 deficiency by analysis of urinary pterins profile , BH4 loading test , and determination of dihydropteridine reductase in RBC .

所有患者均经尿蝶呤谱分析、BH4口服负荷试验及红细胞二氢蝶呤还原酶测定，确诊为BH4缺乏症。

The results also demonstrated that TEA was the best additive , which not only inhibited effectively the corrosion of Ni in alkaline medium , but also promoted the electrochemical oxidation of BH4 – .

研究还结果表明：TEA添加剂的效果最好，不仅能有效抑制Ni金属的腐蚀，还能促进BH4–的电化学氧化。

Objective Tetrahydrobiopterin ( BH4 ) is known to normalize blood phenylalanine levels in BH4 deficiency , but not in phenylketonuria ( PKU ) .

目的四氢生物蝶呤（BH4）可以使BH4缺乏症病人的血液苯丙氨酸水平正常化，但是对苯丙酮酸尿症（PKU）病人无效。

Disorder is typically characterized by low levels of the neurotransmitter metabolite homovanillic acid and reduced levels of neopterin and tetrahydrobiopterin ( BH4 ) in the cerebrospinal fluid .

典型病例脑脊液中神经递质的代谢产物高香草酸和新喋昤及四氢生物喋呤（BH4）的水平下降。

Six single base mutations were detected in 18 unrelated northern Chinese BH4 deficiency families , and the mutations at nucleotides 259C → T and 286G → A were common mutations .

对18例BH4缺乏症患者及其家系进行基因分析，发现6种基因突变，其中259C→T和286G→A为中国北方人的常见突变。

Methods One hundred patients with unknown motor disturbance and mental retardation were referred to this study . All patients were performed by phenylalanine ( Phe ) and BH4 loading test , urinary pterin analysis and dihydropteridine reductase ( DHPR ) activity .

方法对100例运动及智能障碍患者进行苯丙氨酸（phenylalanine，Phe）及BH4负荷试验、尿蝶呤谱分析、红细胞二氢蝶啶还原酶测定，并对部分患者进行多巴治疗性诊断；

This study aimed to investigate the effect of BH4 and phenylalanine hydroxylase ( PAH ) gene mutations in patients with BH4 responsive mild PKU and mild hyperphenylalaninemia ( HPA ) . Compared with W. Mainly discusses the architecture and principle of the .

本研究将探讨BH4和苯丙氨酸羟化酶（PAH）基因突变在对BH4有反应的轻度PKU和轻度高苯丙酸血症（HPA）患者中的作用。

There are B-H bond , H-H bond and atom-molecular bond in BH + 4 and BH4 . There are four equivalent B-H bonds in BH-4 . In the case of BH4 there is an unpaired electron that occurs near the boron atom .

BH4+和BH4中都存在BH键、HH键和原子-分子键；而BH4-中存在着四个相同的BH键；