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Total sulfur amino acid requirement in young men as determined by indicator amino acid oxidation with L-[1-13C]phenylalanine
amino acid requirement stable isotopes phenylalanine
2016/6/14
Determining the sulfur amino acid (SAA) requirements
of humans has remained elusive because of the complex
nature of SAA metabolism. Current recommendations are based
on nitrogen balance studies.
Intake of major nutrients by women in the Maternal Phenylketonuria (MPKU) Study and effects on plasma phenylalanine concentrations
genotype plasma phenylalanine protein
2016/6/13
Women with untreated phenylketonuria (PKU)
often have poor reproductive outcomes.
Tyrosine requirement of healthy men receiving a fixed phenylalanine intake determined by using indicator amino acid oxidation
stable isotopes adult amino acid requirements Amino acid metabolism
2016/6/12
The currently accepted total aromatic amino acid
requirement for adults is based on nitrogen balance measurements
in individuals who received their intake of aromatic amino
acids solely as phenylal...
Phenylketonuria: tyrosine supplementation in phenylalanine-restricted diets
Phenylketonuria PKU tyrosine
2016/6/12
Treatment of phenylketonuria (PKU) consists
of restriction of natural protein and provision of a protein substitute
that lacks phenylalanine but is enriched in tyrosine. Large
and unexplained diffe...
苯丙氨酸羟化酶（PAH）基因外显子7及其两侧内含子的突变研究Mutations in Exon 7 of the Phenylalanine Hydroxylase（PAH）Gene in Chinese Patients with Phenylketonuria
苯丙氨酸羟化酶 外显子 基因突变 单链构象多态性 序列分析
2008/1/6
为探讨中国苯丙酮尿症（PKU）人群中苯丙氨酸羟化酶（PAH）基因外显子7的突变特征，对147例PKU患儿的294个PAH基因外显子7以及两侧部分内含子序列，应用PCR－单链构象多态性（SSCP）分析及基因序列分析的方法进行了筛查和确定。共发现13种突变基因：G239D、R241C、R241fs、R243Q、G247S、G247V、R252Q、L255S、R261Q、M276K、E280G、P281...
Threonine requirement of young men determined by indicator amino acid oxidation with use of L-[1-13C]phenylalanine
Threonine indicator amino acid oxidation, amino acid requirement stable isotope phenylalanine men
2016/6/3
Background: Threonine is an indispensable amino acid with a
complex degradative pathway. Use of the indicator amino acid
oxidation technique should provide an estimate of the threonine
requirement ...