phosphofructokinase


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phosphofructokinase

[‚fäs·fə‚fru̇k‚tō′kī‚nās]
(biochemistry)
A glycolytic enzyme that functions in carbohydrate metabolism by catalyzing the phosphorylation of fructose phosphate.
References in periodicals archive ?
Vora S, Davidson M, Seamon C, et al: Heterogeneity of the molecular lesions in inherited phosphofructokinase deficiency.
Tarui S, Okuno G, Ikura Y, et al: Phosphofructokinase deficiency in skeletal muscle.
Raben N, Sherman JB, Adams E, et al: Various classes of mutations in patients with phosphofructokinase deficiency (Tarui's disease).
Ronquist G, Rudolphi G, Engstrom I, et al: Familial phosphofructokinase deficiency is associated with a disturbed calcium homeostasis in erythrocytes.
Massa R, Sancesario G, Bernardi G: Muscle phosphofructokinase deficiency.
ALTHOUGH about 40 families with autosomal recessive muscle phosphofructokinase deficiency (glycogenosis type VII, Tarui's disease) have been reported, (1) little is known about the long-term course and progression of the disease.
4) Phosphofructokinase is tetrameric under the control of 3 loci that encode for 3 distinct subunits (M, L, P).
Lin HC, Young C, Wang PJ, et al: Muscle phosphofructokinase deficiency (Tarui's disease): report of a case.
Amit R, Bashan N, Abarbanel JM, et al: Fatal familial infantile glycogen storage disease: multisystem phosphofructokinase deficiency.
Vissing J, Galbo H, Haller RG: Paradoxically enhanced glucose production during exercise in humans with blocked glycolysis caused by muscle phosphofructokinase deficiency.
org 1,2,4,5,8; Spanish materials MULTIPLE SULFATASE DEFICIENCY See: Hearing Impairments; Ichthyosis; Leukodystrophy; Tay-Sachs Disease MUSCLE PHOSPHOFRUCTOKINASE DEFICIENCY See: Glycogen Storage Diseases; Muscular Dystrophy MUSCLE PHOSPHORYLASE DEFICIENCY See: Glycogen Storage Diseases; Muscular Dystrophy MUSCULAR ATROPHY, PERONEAL See: Charcot-Marie-Tooth Disease MUSCULAR DYSTROPHY See also: Facio-Scapulo-Humeral Muscular Dystrophy; Ventilator Use Duchenne Parent Project 4785 Emerald Way Middletown, OH 45044 (800) 714-KIDS (5437) (513) 424-0696 (513) 425-9907(fax) ?
Developmental profiles for the activity, concentration, and molecular structure of the pyrophosphate and ATP-dependent phosphofructokinases, phosphoenolpyruvate carboxylase and pyruvate kinase.

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