-transparent.png){kind=logo}
Increased concentrations of total sulfated mucopolysaccharides (MPS), threefold, and hyaluronic acid (HA), 10-fold, were found in ML IV fibroblast extracts when compared to normal controls. Such accumulations altered the distribution of MPS:HA comprised 70% of total MPS in ML IV but only 30% in control cells. Intracellular sulfated MPS was observed accumulating almost linearly in ML IV fibroblasts. "Pulse-chase" experiments indicate that both HA and the sulfated MPS remain in the ML IV cells for long periods of time; in control cells, they are rapidly removed as low molecular weight, dialyzable fragments. These data suggest that the MPS accumulation in ML IV fibroblasts, is the consequence of a catabolic block, probably involving the lysosome.
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
October 1975,
Biochemical and biophysical research communications,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
January 1988,
Journal of inherited metabolic disease,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
June 1995,
Pediatric research,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
January 1988,
Enzyme,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
January 1976,
Experimental cell biology,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
January 1980,
Biochemical and biophysical research communications,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
September 1985,
Archives of biochemistry and biophysics,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
June 1986,
Clinica chimica acta; international journal of clinical chemistry,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
November 1972,
The Biochemical journal,
G Bach, and
M Ziegler, and
G Kohn, and
M M Cohen
June 1992,
Biochimica et biophysica acta,
