Hereditary and Acquired Erythrocyte Phosphofructokinase Deficiency
スポンサーリンク
概要
- 論文の詳細を見る
The control mechanism of glycolysis in human erythrocytes was analysed placing an emphasis upon the relationship between phosphofructokinase (PFK) activity and 2, 3-bisphosphoglycerate (2, 3-DPG) level. The blood sample was directly taken into a syringe containing the perchloric acid in order to minimize the changes in metabolite levels after venopuncture. Alterations in glycolysis in erythrocytes in the patients with hereditary eythrocyte PFK deficiency (PFK activity was 30% of normal control), diabetic ketoacidosis and chronic renal failure in acidosis were investigated by the enzymatic analysis of glycolytic intermediates and adenine nucleotides. The following results were obtained.<BR>1) In PFK deficiency, the levels of glucose-6-phosphate (G6P) and fructose-6-phosphate (F6P) were increased, while those of fructose-1, 6-bisphosphate (FDP), dihydroxyacetone phosphate (DHAP) plus glyceraldehyde-3-phosphate (GAP) and 2, 3-DPG were decreased.<BR>2) In diabetic ketoacidosis, the level of F6P was significantly higher than normal control and levels of FDP, DHAP plus GAP, 2, 3-DPG and 3-phosphoglycerate were significantly lowered. In chronic renal failure, the similar patterns in the levels of glycolytic intermediates were demonstrated when the levels were compared with those of the non-azotemic patients with comparable anemia and reticulocytosis.<BR>These findings indicate that glycolysis in erythrocytes in these genetic and aquired diseases is inhibited at PFK step. It was demonstrated in experiments using human erythrocyte suspension and purified PFK preparations that the inhibition in acidosis was mainly due to the increased hydrogen ion concentration. Thus, a reduction in erythrocyte PFK activity is caused not only by the hereditary defect of enzyme protein but also by the elevated concentration of hydrogen ion in blood. The marked fall in 2, 3-DPG level is caused by the reduction of PFK activity in erythrocytes, indicating an important role of this enzyme in the regulation of the 2, 3-DPG level.
- Japan Society of Clinical Chemistryの論文
著者
関連論文
- 集団検診における痛風, 無症候性高尿酸血症の経時的変化 : 3年間の縦断的観察
- イノシンとヒポキサンチン
- 糖尿病眼合併症をもつ患者に対するチーム医療と患者教育に関する実態調査II : 教育に携わる看護婦の現状
- 糖尿病眼合併症をもつ患者に対するチーム医療と患者教育に関する実態調査I : 糖尿病患者教育に対する医療チームの現状
- 司会のことば
- 甲状腺機能低下性ミオパチーにおける運動筋のプリン体分解亢進について
- 代謝疾患
- わが国における先天性代謝異常症の出生前診断の現状
- 筋ホスホフルクトキナーゼ欠損症 : 1家系に由来する3症例について
- 家族性地中海熱型を呈しコルヒチンが奏効した周期熱の1例
- Congenital dyserythropoietic anemia type Iの1症例
- 肝障害に伴う高アンモニア血症に対する血中アンモニア測定簡易キット法の臨床的応用
- 著明な両側性肺動脈血栓を呈したBehçet病の1例
- 著明な脳萎縮に合併したADH分泌異常症候群(SIADH)の1例
- 前頚部巨大腫瘤を主症状としたサルコイドーシスの1例
- 腎性低尿酸血症を有し再発性下腿潰瘍に自家植皮術が有効であった48, XXYY症候群の1例
- 保存的治療により治癒した脾内膵仮性嚢胞の1例
- 特発性血小板減少性紫斑病の経過中に重症筋無力症を合併した1症例
- Phosphofructokinase lsozyme: Studies on Liver Phosphofructokinase
- ラツト灌流膵におけるXenopsin, Neurotensin, Substance Pのグルカゴン分泌能
- 長寿家系に発生した家族性高コレステロ-ル血症homozygoteの1例
- Hyperlipoproteinemia in primary gout (Second report):Qualitative abnormalities in low-density lipoproteins from gouty patients
- A case of apolipoprotein E3 deficiency (E2/2 phenotype) associated with renovascular hypertension and atherosclerosis obliterans.
- 4. 膵内分泌
- タイトル無し
- Alterations of urinary excretion of antipyrine metabolites in patients with cirrhosis of the liver.
- Two brothers with hereditary xanthinuria due to xanthine oxidase deficiency
- Molecular biological approaches to the mechanism of myogenic hyperuricemia: analysis of the primary structure and the genomic structure of muscle - type isozyme of human phosphofructokinase
- A family of hereditary xanthinuria: Two siblings with peptic ulcer and hypouricemia due to xanthine oxidase deficinecy, and a heterozygote(father) with gout.
- Hypouricemia associated with hyperoxypurinemia due to altered renal handling - Analysis by probenecid - and pyrazinamide - loading tests
- Purine Degradation in Contracting Fast and Slow Muscle
- Enhanced purine degradation in muscles of patients with hypoparathyroidism
- Effect of Parathyroid Function on the Uric Acid Metabolism in a Case of Primary Gout Associated with Parathyroid Adenoma and Renal Cell Carcinoma
- A study on the distribution of 3-methylcholanthrene-inducible cytochrome P-450 within the hepatic lobulus of rats.
- Studies on the Abnormal Metabolism of Uric Acid in Massively Obese Patients:Remarkable Impairment of Renal Urate Excretion and Its Improvement by Low - calorie Diet
- The effect of an .ALPHA.-glucosidase inhibitor on fatty liver in obesity.
- Studies on the Metabolism of Purine Nucleotide in Massive Obese Subjects:(I) Changes of Serum Uric Acid during Intensive Weight Reduction
- Effect of Daily Consumption Dose of Ethanol on the Ethanol Induced Hyperuricemia
- A Contribution of Hepatic Purine Degradation Induced by Hypoglycemia to the Development of Hyperuricemia in Glycogen Storage Disease Type I
- Treatment of myogenic hyperuricemia:Effects of glucose infusion and allopurinol administration on purine metabolism in patients with muscular glycogenoses
- Hereditary and Acquired Erythrocyte Phosphofructokinase Deficiency
- Metabolic Disturbance in Phosphofructokinase Deficient Erythrocytes of Glycogenosis VII
- Diabetes Mellitus and2, 3-DPG
- A study of the role of cytochrome b5 in the reductive metabolism of halothane.
- A case of renal hypouricemia associated with increased plasma oxypurine concentration due to its decreased excretion
- Overproduction of oxypurines through myogenic and hepatogenic mechanisms
- Pathogenesis of Hyperuricemia in Type VII Glycogenosis:an accelerated purine degradation in exercising muscle
- A case of renal hypouricemia with Parkinsonism
- Approach to the hexosamine metabolism
- Diabetic Microangiopathy and Metabolism of Glycosides
- Studies on the lipid and lipoprotein levels in gout and asymptomatic hyperuricemia -The possible role of serum apoprotein B containing lipoproteins in preventing the occurrence of gouty attack
- Systematic Diagnosis of Myopathy due to Muscle Glycogenosis
- Molecular Basis of Myogenic Hyperuricemia:Analysis of Tissue-specific Expression of Phosphofructokinase-M Gene Transcripts
- A Case of Renal Hypouricemia Associated with XXYY Syndrome
- Treatment of Myogenic Hyperuricemia:Allopurinol Effects in Muscle Phosphofructokinase Deficiency
- A Case of Hypouricemia due to Combined Reabsorption Defect
- Three cases of renal hypouricemia
- Hyperuricemia associated with familial hypercholesterolemia
- A case of mesenteric gastrinoma identified six years after diagnosis of Zollinger-Ellison syndrome.
- Family study of hereditary xanthine (xanthine oxidase deficiency ):increased urinary excretions of xanthine and hypoxanthine in heterozygotes and association with gout in a heterozygote
- タイトル無し
- Hexosamine Metabolism and Diabetes Mellitus
- Exercise- induced alteration of erythrocyte glycolysis -Its relation to accelerated degradation of muscular adenine nucleotides
- Protective effect of camostat mesilate and allopurinol on acute edematous pancreatitis in the rat induced by excessive doses of caerulein.
- Two cases of chronic idiopathic intestinal pseudoobstruction with abnormal esophageal motility and bilateral hydronephrosis.
- :EVALUATION OF AORTO-CORONARY BYPASS SURGERY
- Lipidbiochemical Characterization of Various Kinds of Lysosomes
- Gastrointestinal Glucagon in Pathophysiology of Diabetes Mellitus
- Pathogenesis of Hyperuricemia in Glycogenosis Type VII:a contribution of an exaggerated degradation of purine nucleotides in exercising muscle
- Adult Case of Glycogenosis Type I with Hyperuricemia
- Myogenic Hyperuricemia: a novel mechanism of hyperuricemia found in type VII glycogenosis
- A case of allopurinol - induced fever myositis and dermatitis
- Hyperlipoproteinemia in Primary Gout with Respect to Phenotypes of Hyperlipoproteinemia
- Enzyme Defects in Erythrocytes
- Symposium on recent trend in the research of hyperlipidemia in Japan. (1). Profiles of primary hyperlipidemia in Japan.