Lysosomal di-N-acetylchitobiase-deficient mouse tissues accumulate Man2GlcNAc2 and Man3GlcNAc2
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چکیده
منابع مشابه
Structure of the human gene for lysosomal di-N-acetylchitobiase.
Chitobiase is a lysosomal glycosidase that acts during the ordered degradation of asparagine-linked glycoproteins to cleave the core chitobiose unit at its reducing end. Human chitobiase is expressed in significant amounts, while bovine chitobiase is produced at extremely low levels. To begin to understand this species-dependent expression, we determined the gene structure of human chitobiase. ...
متن کاملA di-N-acetylchitobiase activity is involved in the lysosomal catabolism of asparagine-linked glycoproteins in rat liver.
A perfused rat liver was used to study the effects of 5-diazo-4-oxo-L-norvaline on lysosomal glycoprotein catabolism. Addition of this compound (1.0 mM) to the perfusate reduced activity of beta-aspartyl-N-acetylglucosylamine amidohydrolase by 99% in 1 h. Treated livers were unable to completely degrade endocytosed N-acetyl[14C]glucosamine-labeled asialo-alpha 1-acid glycoprotein as evidenced b...
متن کاملOptimum substrate size and specific anomer requirements for the reducing-end glycoside hydrolase di-N-acetylchitobiase.
Di-N-acetylchitobiase is a family 18 glycoside hydrolase that splits the reducing-end GlcNAc from chitooligosaccharides. The enzyme hydrolyzed only the alpha-anomer of five tested substrates, chitin di- through hexasaccharide. In all cases the glycosyl fragment retained its beta-configuration while the split monosaccharide was alpha-D-GlcNAc. Chitobiose was hydrolyzed less than half as fast as ...
متن کاملUnprocessed myogenin transcripts accumulate during mouse embryogenesis.
The gene myogenin encodes a helix-loop-helix protein whose function is critical to the integrity of the mammalian myogenic cascade. In vitro, the expression of this gene immediately precedes terminal differentiation, as measured by the synthesis of those proteins that make up the contractile apparatus. However, during mammalian development, expression of myogenin and the appearance of sarcomere...
متن کاملN,N′-Di-8-quinolyladipamide
The complete molecule of the title compound, C(24)H(22)N(4)O(2), is generated by a crystallographic inversion centre located at the mid-point of the central C-C bond. The quinoline ring system and the hexyl chain are both essentially planar, and the dihedral angle between them is 46.30 (2)°. Intra-molecular N-H⋯N and C-H⋯O hydrogen bonds form five- and six-numbered rings, respectively. The crys...
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ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease
سال: 2012
ISSN: 0925-4439
DOI: 10.1016/j.bbadis.2012.03.005