Physiological Substrates for Human Lysosomal β-Hexosaminidase S
نویسندگان
چکیده
منابع مشابه
Immunochemical and biochemical investigation of hexosaminidase S.
Hexosaminidase S (HEX S), the residual isozyme found in tissues and body fluids of children with the O variant of GM2 gangliosidosis, was purified from tissues of variant individuals and biochemically and immunochemically characterized. This enzyme has an apparent molecular weight of 103,000 with an isoelectric point of 4.2, is heat labile to the same extent as HEX A, and loses most of its acti...
متن کاملTherapeutic Potential of Intracerebroventricular Replacement of Modified Human β-Hexosaminidase B for GM2 Gangliosidosis
To develop a novel enzyme replacement therapy for neurodegenerative Tay-Sachs disease (TSD) and Sandhoff disease (SD), which are caused by deficiency of β-hexosaminidase (Hex) A, we designed a genetically engineered HEXB encoding the chimeric human β-subunit containing partial amino acid sequence of the α-subunit by structure-based homology modeling. We succeeded in producing the modified HexB ...
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Monocyte hydrolases are harmful when secreted inappropriately. In this study we have investigated the levels of one of the hydrolases. beta-hexosaminidase in patients with obstructive jaundice. These patients showed markedly elevated plasma levels, and their monocytes show increased spontaneous secretion and total enzyme content. The plasma enzyme levels correlate with monocyte enzyme content a...
متن کاملRole for lysosomal enzyme beta-hexosaminidase in the control of mycobacteria infection.
The pathogenic mycobacteria that cause tuberculosis (TB) and TB-like diseases in humans and animals elude sterilizing immunity by residing within an intracellular niche in host macrophages, where they are protected from microbicidal attack. Recent studies have emphasized microbial mechanisms for evasion of host defense; less is known about mycobactericidal mechanisms that remain intact during i...
متن کاملN-acetyl-β-hexosaminidase in chronic tonsillitis and tonsillar hypertrophy.
BACKGROUND The concentration and specific activity of N-acetyl-β-hexosaminidase (HEX) in palatine tonsils with chronic tonsillitis and tonsillar hypertrophy give insight in tonsillar tissue remodeling and constitute a potential marker for diagnosis and treatment of chronic tonsillitis and tonsillar hypertrophy. AIM Determining the concentration and specific activity of N-acetyl-β-hexosaminida...
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ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2002
ISSN: 0021-9258
DOI: 10.1074/jbc.m105457200