Therapeutic Use of Low Phenylalanine Peptide Milk for Phenylketonuria

低苯丙氨酸肽奶治疗苯丙酮尿症的用途

基本信息

  • 批准号:
    59870037
  • 负责人:
  • 金额:
    $ 18.62万
  • 依托单位:
  • 依托单位国家:
    日本
  • 项目类别:
    Grant-in-Aid for Developmental Scientific Research
  • 财政年份:
    1984
  • 资助国家:
    日本
  • 起止时间:
    1984 至 1986
  • 项目状态:
    已结题

项目摘要

Low-phenylalanine (Phe) milk with amino acids as a nitrogen source is currently in common use for the treatment of phenylketonuria (PKU), however, more than a few of the patients with PKU dislike these special dietary products because of the bad taste and odor peculiar to some of the amino acids contained in them. The aim of our study is that a more palatable and dietric milk is prepared by using a low-Phe peptide as a protein source, because it has been reported that peptides do not have the unpleasant odor and are more easily aborbed.Whey protein was used as the protein source. Pepsin was used to obtain the peptide containing phenylalanine (Phe) as the C-terminal and carboxypeptidase was used to shorten the peptide chain and to remove the Phe terminal. The hydrolysate was fractionated by a cellulofine GH-25m or charcoal column to obtain the low Phe peptides. Low-Phe peptide (LPP) milk was made at Yukijirushi (Snow Bland) Milk Manufacturing Co., Ltd.Thirty-two normal healthy subjects … More and 12 children with PKU aged 11 years or over reported that they found the product less palatable than ordinary powdered milk for infant feeding but the product more palatable than the milk formula with amino acid powders as the main constituent. On the other hand, PKU children aged 3-10 years who had long been on amino acid-based milk formulas for PKU had no liking for the LPP-milk simply because they had gotten used to the taste and odor peculiar to amino acid powders. They could set used to the LPP product as well, chould they be placed on it early enough in life. Actually, both LPP-milk and low-Phe milk with amino acid mixture are acceptable to PKU infants aged 2 years or less because their sense of taste is still immature. Finally, LPP-milk has been used on 6 infants with PKU and a normally developed female PKU, who wants to become pregnant, for more than a year with great effectiveness in reducing the the serum Phe concentrations. Diarrhea is less frequent in the infants on LPP-milk than those on milk with amino acid mixture because the former has a lower osmolality than later. Less
以氨基酸为氮源的低苯丙氨酸(Phe)牛奶是目前治疗苯丙酮尿症(PKU)的常用方法,但有相当一部分PKU患者不喜欢这些特殊的饮食产品,因为其中的一些氨基酸具有难闻的味道和气味。我们研究的目的是用低PHE的多肽作为蛋白质来源来制备更美味和营养丰富的牛奶,因为据报道,多肽没有难闻的气味,更容易流产。以胃酶为底物,得到以苯丙氨酸(Phe)为C端的多肽,用羧基肽酶缩短多肽链,去除Phe末端。水解物经纤维素GH-25M或木炭柱分离,得到低Phe多肽。低Phe多肽(LPP)牛奶由日本雪域乳业有限公司生产。32名正常健康受试者…超过12名11岁或以上的北京大学儿童表示,他们认为该产品不如普通奶粉适合婴儿喂养,但比以氨基酸粉为主要成分的配方奶粉更可口。另一方面,长期服用北大氨基酸配方奶粉的北大3-10岁儿童不喜欢LPP奶粉,因为他们已经习惯了氨基酸粉特有的味道和气味。他们也可以习惯LPP产品,如果他们在生命中足够早地被放在上面的话。事实上,LPP牛奶和低Phe氨基酸混合物牛奶对于2岁及以下的PKU婴儿来说都是可以接受的,因为他们的味觉还不成熟。最后,LPP-牛奶已用于6例PKU婴儿和1例发育正常的女性PKU,一年多来,在降低血清Phe浓度方面取得了很好的效果。服用LPP-牛奶的婴儿腹泻的发生率低于服用氨基酸混合物牛奶的婴儿,因为前者的渗透压低于后者。较少

项目成果

期刊论文数量(19)
专著数量(0)
科研奖励数量(0)
会议论文数量(0)
专利数量(0)
小児科臨床. 38-4. (1985)
儿科临床实践。38-4。
  • DOI:
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    0
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  • 通讯作者:
吉田泰祥: 小児内科. 18. 1169-1175 (1986)
吉田康义:小儿内科。18。1169-1175(1986)
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    0
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北川照男: "新内科学大系年刊版'85-B(先天性代謝異常症の予防と治療)" 中山書店, 183-207 (1985)
北川辉夫:《新内科年刊85-B(先天性代谢紊乱的预防和治疗)》中山书店,183-207(1985)
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    0
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新内科学大系. 年刊版 '85-B. (1985)
新内科系统。年度版85-B(1985)。
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  • 影响因子:
    0
  • 作者:
  • 通讯作者:
Akiko Maeda: Agric.Biol.Chem. 51. (1987)
前田明子:农业、生物、化学。
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KITAGAWA Teruo其他文献

KITAGAWA Teruo的其他文献

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{{ truncateString('KITAGAWA Teruo', 18)}}的其他基金

Treatment of Inherited Metabolic Diseases by Bone Marrow Transplantation
骨髓移植治疗遗传性代谢性疾病
  • 批准号:
    63480241
  • 财政年份:
    1988
  • 资助金额:
    $ 18.62万
  • 项目类别:
    Grant-in-Aid for General Scientific Research (B)
Study on the Development of the Therapeutic Formula for Inborn Errors of Amino Acid Metabolism Consisting of Synthetic Peptide as a Nitrogen Source
以合成肽为氮源的先天性氨基酸代谢缺陷治疗配方的研制
  • 批准号:
    62870040
  • 财政年份:
    1987
  • 资助金额:
    $ 18.62万
  • 项目类别:
    Grant-in-Aid for Developmental Scientific Research
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