Poly(ADP-ribose) metabolism is altered in cells from a patient with glutamyl ribose-5-phosphate storage disease

Conference · Thu May 01 04:00:00 EDT 1986 · Fed. Proc., Fed. Am. Soc. Exp. Biol.; (United States)

OSTI ID:5204569

Jacobson, E L; Gupta, G; Williams, J C; Jacobson, M K

Glutamyl Ribose 5-Phosphate Storage Disease (GRPSD) has been suggested to result from a defect in the metabolism of ADP-ribosylated proteins. The authors have compared the response to N-methyl-N'-nitro-N-nitrosoguanidine (MNNG) of cells from a patient with GRPSD to age-matched control cells. GRPSD cells exhibited a normal cytotoxic response to a single challenge of MNNG. The NAD pools of confluent cultures were prelabelled with /sup 3/H-adenine and time courses of NAD and poly(ADP-ribose) content were determined following varying doses of MNNG using newly developed methodologies. GRPSD cells showed a greatly reduced accumulation of poly(ADP-ribose) relative to controls. Likewise, the rate of depletion of NAD was also decreased several fold following MNNG treatment. GRSPD cells should prove valuable in the study of the role of poly(ADP-ribose) in cellular recovery from DNA damage.

• Research Organization: North Texas State Univ., Fort Worth
• OSTI ID: 5204569
• Report Number(s): CONF-8606151-
• Conference Information: Journal Name: Fed. Proc., Fed. Am. Soc. Exp. Biol.; (United States) Journal Volume: 45:6
• Country of Publication: United States
• Language: English

Related Subjects

550901* -- Pathology-- Tracer Techniques
59 BASIC BIOLOGICAL SCIENCES
ADP
ALDEHYDES
CARBOHYDRATES
COENZYMES
DIGESTIVE SYSTEM DISEASES
DISEASES
ISOTOPE APPLICATIONS
LABELLED COMPOUNDS
METABOLISM
MONOSACCHARIDES
NAD
NUCLEOTIDES
ORGANIC COMPOUNDS
PATHOGENESIS
PATIENTS
PENTOSES
RIBOSE
SACCHARIDES
TRACER TECHNIQUES
TRITIUM COMPOUNDS