Developmental changes of 6-phosphofructo-1-kinase subunit levels in erythrocytes from normal dogs and dogs affected by glycogen storage disease type VII.
1. The subunit proportions (L:M:C) of the PFK isozymes from normal adult erythrocytes were 2:86:12. Affected adult erythrocyte 6-phosphofructo-1-kinase (PFK) isozymes contained normal L-type (31%) and C-type (61%) subunits as well as a small amount (8%) of truncated M-type subunit. 2. When measured within 24 hr of birth, both normal and affected dog erythrocytes contained high PFK activities due to elevated levels of the L-type subunit. As the dogs matured, PFK activity decreased due to a greater than 99% loss of the L-type subunit. 3. By 2 weeks of age, the M-type and C-type subunits in normal dog PFK isozymes increased several-fold and attained near adult levels. 4. During post-natal development, the L-type subunit from affected dog erythrocytes decreased more rapidly than from normal dog erythrocytes; but it was maintained at a higher level in the affected adult erythrocytes. Also, in the affected dog erythrocytes, truncated M-type subunits were detected; and the initially high levels of the C-type subunit decreased approximately 50% after 4 weeks.
Mhaskar
Y
Y
Department of Pharmacology, Southern Illinois University School of Medicine, Springfield 62794-9230.
Harvey
J W
JW
Dunaway
G A
GA
D000375:Aging / Q000378:metabolism; D000818:Animals; D004195:Disease Models, Animal; D004285:Dogs; D004912:Erythrocytes / Q000201:enzymology*; D006014:Glycogen Storage Disease Type VII / Q000201:enzymology*; D015151:Immunoblotting; D010732:Phosphofructokinase-1 / Q000378:metabolism*; D012016:Reference Values
D010732:Phosphofructokinase-1