Developmental changes of 6-phosphofructo-1-kinase subunit levels in erythrocytes from normal dogs and dogs affected by glycogen storage disease type VII.

Abstract

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.

• Authors: Y Mhaskar, J W Harvey, G A Dunaway
• Journal: Comparative biochemistry and physiology. B, Comparative biochemistry (Comp Biochem Physiol B) Vol. 101 Issue 3 Pg. 303-7 (Mar 1992) ISSN: 0305-0491 [Print] England
• PMID: 1533828 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)

Chemical References

• Phosphofructokinase-1

Topics

• Aging (metabolism)
• Animals
• Disease Models, Animal
• Dogs
• Erythrocytes (enzymology)
• Glycogen Storage Disease Type VII (enzymology)
• Immunoblotting
• Phosphofructokinase-1 (metabolism)
• Reference Values