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从两名α-N-乙酰半乳糖胺酶活性遗传性缺乏患者的尿液中分离并对O-糖苷型含唾液酸糖肽进行结构表征。

Isolation and structural characterization of sialic-acid-containing glycopeptides of the O-glycosidic type from the urine of two patients with an hereditary deficiency in alpha-N-acetylgalactosaminidase activity.

作者信息

Linden H U, Klein R A, Egge H, Peter-Katalinic J, Dabrowski J, Schindler D

机构信息

Institut für Physiologische Chemie, Universität Bonn.

出版信息

Biol Chem Hoppe Seyler. 1989 Jul;370(7):661-72. doi: 10.1515/bchm3.1989.370.2.661.

DOI:10.1515/bchm3.1989.370.2.661
PMID:2775488
Abstract

Glycopeptides have been isolated from the urine of two patients, aged 5 and 6, with a new lysosomal storage disease characterized by a deficiency in alpha-N-acetylgalactosaminidase activity. Isolation of these glycopeptides was achieved using gel filtration and ion-exchange chromatography. Structural determination was done using one- and two-dimensional 500 MHz 1H-NMR spectroscopy and FAB mass spectrometry of native and derivatized glycopeptides. The following structures were inferred as being present: Glycopeptide A (up to 140 mg/l urine) (1)-(3) Neu5Ac alpha 2-3Gal beta 1-3 (Neu5Ac alpha 2-6)GalNAc alpha 1-R A1: R = Ser A2: R = Thr A3: R = Thr-Pro Glycopeptide B (up to 80 mg/l urine) (4)-(6) Neu5Ac alpha 2-3Gal beta 1-4GlcNAc beta 1-6 (Neu5Ac alpha 2-3-Gal beta 1-3) GalNAc alpha 1-R B1: R = Ser B2:R = Thr B3: R = Thr-Pro

摘要

已从两名分别为5岁和6岁的患有一种新型溶酶体贮积病的患者尿液中分离出糖肽，该疾病的特征是α-N-乙酰半乳糖胺酶活性缺乏。采用凝胶过滤和离子交换色谱法实现了这些糖肽的分离。利用一维和二维500兆赫1H-NMR光谱以及天然和衍生化糖肽的快原子轰击质谱法进行了结构测定。推断存在以下结构：糖肽A（尿液中含量高达140毫克/升）(1)-(3) Neu5Acα2-3Galβ1-3(Neu5Acα2-6)GalNAcα1-R A1：R = Ser A2：R = Thr A3：R = Thr-Pro 糖肽B（尿液中含量高达80毫克/升）(4)-(6) Neu5Acα2-3Galβ1-4GlcNAcβ1-6(Neu5Acα2-3-Galβ1-3)GalNAcα1-R B1：R = Ser B2：R = Thr B3：R = Thr-Pro

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J Med Genet. 1996 Jun;33(6):458-64. doi: 10.1136/jmg.33.6.458.
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