| Abnormal high-energy phosphate metabolism in human muscle phosphofructokinase deficiency. | |
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MedLine Citation:
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PMID: 1827789 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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We studied the pattern of high-energy phosphate metabolism in five patients with phosphofructokinase deficiency (PFKD) and five healthy subjects (HS) during graded rhythmic handgrip performed for 5 min at 17, 33, 50, and 100% of maximal voluntary contraction (MVC). The range of MVC was similar in both groups. Force production was recorded, and intracellular concentrations of phosphorus compounds and pH were measured in the flexor digitorum profundus of the active forearm. At exercise intensities greater than or equal to 50% MVC, changes in concentrations of high-energy phosphate metabolites were abnormal in PFKD. During maximal effort, [ADP], calculated from the creatine kinase reaction, was 64.3 +/- 13.5 (SE) mumol/kg in PFKD vs. 25.7 +/- 4.0 in HS (P less than 0.05). Ammonia (NH3), a product of AMP deamination and an index of muscle [AMP], increased approximately twofold more in venous effluent during maximal forearm exercise in PFKD than in HS (P less than 0.05). Phosphocreatine concentration was 9.4 +/- 1.3 (SE) mmol/kg in HS and 13.0 +/- 1.7 in PFKD (P less than 0.05). Inorganic phosphate concentration was 15.8 +/- 1.4 mmol/kg in HS and 7.4 +/- 0.5 in PFKD (P less than 0.05). During strenuous exercise, PFKD patients exhibit an impairment in the rephosphorylation of ADP related to a subnormal oxidative capacity, an absence of glycolysis, and an attenuated breakdown of phosphocreatine. |
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Authors:
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L A Bertocci; R G Haller; S F Lewis; J L Fleckenstein; R L Nunnally |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, Non-P.H.S.; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: Journal of applied physiology (Bethesda, Md. : 1985) Volume: 70 ISSN: 8750-7587 ISO Abbreviation: J. Appl. Physiol. Publication Date: 1991 Mar |
Date Detail:
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Created Date: 1991-06-24 Completed Date: 1991-06-24 Revised Date: 2007-11-14 |
Medline Journal Info:
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Nlm Unique ID: 8502536 Medline TA: J Appl Physiol Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1201-7 Citation Subset: IM |
Affiliation:
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Department of Radiology, University of Texas Southwestern Medical Center, Dallas 75235. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adenosine Diphosphate
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metabolism Adenosine Monophosphate / metabolism Adolescent Adult Female Humans Male Middle Aged Muscle Contraction / physiology Muscles / metabolism* Phosphates / metabolism* Phosphocreatine / metabolism Phosphofructokinase-1 / deficiency* |
| Grant Support | |
ID/Acronym/Agency:
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HL-01581/HL/NHLBI NIH HHS; HL-06296/HL/NHLBI NIH HHS; HL-07360/HL/NHLBI NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Phosphates; 58-64-0/Adenosine Diphosphate; 61-19-8/Adenosine Monophosphate; 67-07-2/Phosphocreatine; EC 2.7.1.11/Phosphofructokinase-1 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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