Fructose 1,6-bisphosphate aldolase (FPA) was recently known as new member of heparin binding proteins and a new method for FPA purification has been proposed (Thanh Van Ta et all, J. Biochem. 125, 554-559,199) by measuring FPA - heparin binding inhibition caused by various glycosaminoglycans (GAGs), affinity of the two isoforms, aldolase A4 and C4, to the GAGs underphysiological ionic conditions was estimated. Among glycosaminoglycans employd, heparin was confirmed to be the unique one that could bind specifically these enzymes. In the lower ionic strength, the affinity order of both FPA isoforms (A4 and C4) to these GAGs appeared as heparin> chondroitin polysulfate> heparin sulfate > dermatan > chondrointin sulfate A > chondroin sulfate C. Employing the same techniques, the affinity of regioselectively desulfated heparins to FPA was estimated. Our results indicated that, among the sulfate groups is heparin, loss of N-sulfate group reduced most significantly the affinity to FPA A4 and C4. This sugests that FPA recognizes a specific heparin structure including the sulfo-amino group at C2 of the glucosamine residue as the vital factor in this interaction.
Fructose-Bisphosphate Aldolase ; Glycosaminoglycans

Fructose 1,6 bisphosphate adolase A and C (FPA A4 and C44) have been found to bind specifically to heparin in physiological ionic strength. The researcher found that activity of FPA was inhibited by heparin. The inhibition of FPA A4 and C4 depend on the degree of sulfation. Inhibition activity FPA of heparin increase directly proportional to molecule lenghth. Kinetic studies showed that: inhibitory mode of heparin on FPA was the linear mixed type inhibition, in which velocity can be driven to zero at high heparin concentration
Kinetics ; Fructose-Bisphosphate Aldolase ; Heparin

It is of great significance to use biosynthesis to transform the inorganic substance formaldehyde into organic sugars. Most important in this process was to find a suitable catalyst combination to achieve the dimerization of formaldehyde. In a recent report, an engineered glycolaldehyde synthase was reported to catalyze this reaction. It could be combined with engineered D-fructose-6-phosphate aldolase, a "one-pot enzyme" method, to synthesize L-xylose using formaldehyde and the conversion rate could reach up to 64%. This process also provides a reference for the synthesis of other sugars. With the increasing consumption of non-renewable resources, it was of great significance to convert formaldehyde into sugar by biosynthesis.
Biocatalysis ; Formaldehyde ; chemistry ; Fructose-Bisphosphate Aldolase ; metabolism ; Xylose ; chemical synthesis

OBJECTIVE: To assess the 10-year cumulative survival outcome of polymyositis (PM) and dermatomyositis (DM) as well as the factors associated with the the outcome. METHODS: Eighty five patients with PM and twenty one patients with DM were diagnosed at our university medical center between 1997 and 2007. Thirty six patients with PM and 13 patients with DM were followed up until death or until the end of January, 2008. Gender, age, AST, ALT, CPK, LDH, ESR, CRP, aldolase, drugs of therapy, combined ILD, and cancer, and duration of remission after therapy were assessed as prognostic factors of death by the Kaplan-Meier curve and Cox regression model. RESULTS: The respective 10-year survival rate for PM and DM was 80.8% (95% confidence interval (CI): 73.3~87.2) and 55.9% (95% CI: 40.7~71.1), respectively. The median survival for PM and DM was 11.3 years (95% CI: 9.8~12.9) and 7.0 years (95% CI: 3.6~10.5), respectively. Compared to DM patients, the subjects with PM had a 167.26 fold (95% CI: 7.59~3683.19) combined ILD adjusted risk of mortality (p<0.05) and no other individual factor reached significance as a predictor of death. However, cancer had a hazard ratio (HR) of 17.00 (95% CI: 1.06~281.79) and 2.45 (95% CI: 0.78~12.45) for death in the PM and DM group, respectively. CONCLUSION: According to an analysis of the survival and its prognostic factors in patients with PM and DM, ILD is a risk factor for mortality in PM and cancer was risk factor for mortality in both PM and DM.
Academic Medical Centers ; Dermatomyositis ; Fructose-Bisphosphate Aldolase ; Humans ; Myositis ; Polymyositis ; Risk Factors ; Survival Rate

Hereditary fructose intolerance is an autosomal recessive disorder that is caused by a deficiency in fructose-1-phosphate aldolase (Aldolase B). Children can present with hypoglycemia, jaundice, elevated liver enzymes and hepatomegaly after intake of dietary fructose. Long-term intake of fructose in undiagnosed patients can result in hepatic failure or renal failure. We experienced a case of hereditary fructose intolerance presenting as recurrent hepatitis-like episodes. Detailed evaluation of her dietary habits revealed her avoidance of sweetened foods and fruits. Genetic analysis of ALDOB revealed that she is a homozygote for a novel frameshifting mutation c[758_759insT]+[758_759insT] (p.[val25 3fsX24]+[val253fsX24]). This report is the first of a Korean patient diagnosed with hereditary fructose intolerance using only molecular testing without undergoing intravenous fructose tolerance test or enzyme assay.
Child ; Enzyme Assays ; Food Habits ; Frameshift Mutation ; Fructose ; Fructose Intolerance ; Fructose-Bisphosphate Aldolase ; Fructosephosphates ; Fruit ; Hepatitis ; Hepatomegaly ; Homozygote ; Humans ; Hypoglycemia ; Jaundice ; Liver ; Liver Failure ; Renal Insufficiency

Proximal muscle weakness can be induced by many diseases, such as muscular dystrophies, inflammatory muscle diseases, and polymyalgia rheumatica. Differential diagnosis of these diseases is important. The patient had proximal muscle weakness with a normal creatine kinase (CK) level. Our initial diagnosis was polymyalgia rheumatica because the CK level was normal. The patient was treated with low-dose corticosteroid. However, the muscle weakness did not improve. The diagnosis of polymyositis was confirmed by a muscle biopsy. We suggest that if the patient has typical symptoms with normal CK, then evaluations for inflammatory muscle diseases are essential.
Biopsy ; Creatine Kinase* ; Creatine* ; Diagnosis ; Diagnosis, Differential ; Fructose-Bisphosphate Aldolase ; Humans ; Muscle Weakness ; Muscles ; Muscular Dystrophies ; Myositis ; Polymyalgia Rheumatica ; Polymyositis*

Dermatomyositis is a rare and idiopathic inflammatory myopathy with a characteristic cutaneous manifestation. A 62-year-old female complained of polyarthralgia that lasted for many years. She was diagnosed with hypomyopathic dermatomyositis by the typical skin rash associated with dermatomyositis but without muscle involvement such as muscle weakness, elevated level of creatinine phosphokinase and aldolase. Her symptoms improved with treatment of hydroxychloroquine and prednisolone. We experienced a case of hypomyopathic dermatomyositis on 62-year-old female patient and report with review of literatures.
Arthralgia ; Creatinine ; Dermatomyositis ; Exanthema ; Female ; Fructose-Bisphosphate Aldolase ; Humans ; Hydroxychloroquine ; Middle Aged ; Muscle Weakness ; Muscles ; Myositis ; Prednisolone

Clinical review has been made for 8 cases of Duchenne muscular dystrophy admitted to orthopedic Department during the time between 1964 and 1969. Duchenne type muscular dystrophy, (Duchenne, 1849), is the most common type of the progressive muscular dystrophy. A number of reports have been found regarding its symptomatology, pathogenesis based on muscle biopsy, heredity and the change of serum enzyme such as aldolase, creatine kinase, and transaminase, though no definite treatment has been known. The results were as follows: 1) All eight cases were male of 5 to 13 years of age. 2) Gowers sign and waddling gait were noted in all cases and contracture in two cases. 3) Familial occurrence was noted in two (Case 1, 2). 4) Urinary creatine value was elevated in all cases, while creatinine excretio decreased in seven cases.
Biopsy ; Contracture ; Creatine ; Creatine Kinase ; Creatinine ; Fructose-Bisphosphate Aldolase ; Gait ; Heredity ; Humans ; Male ; Muscular Dystrophies ; Muscular Dystrophy, Duchenne ; Orthopedics

Bezafibrate, a fibric acid derivative related to clofibrate, is being used increasingly in the treatment of hypertriglycemia. It is relatively well tolerated at usual dosage, and has a low incidence of adverse reactions. But we had recently observed a reversible deterioration of renal function requiring hemodialysis, presumed to be caused by bezafibrate treatment in a patient with diabetic nephropathy. A 55 year old man was admitted with complaints of general weakness and painful lower extremities. He had taken bezafibrate (200 mg every 12 hours) for the previous 4 months because of hypertriglycemia. After admission, the drug was withdrawn, and he was treated conservatively management with hydration and diuretics for bezafibrate induced rhabdomyolysis. Nevertheless, his symptom was not improved, so he was taken even hemodialysis. These findings suggested that bezafibrate should be admistered with great caution to patient with renal insufficiency. When it is admistered, CK, LDH, aldolase, and sGOT levels have to be checked for early detection of potential side effect.
Aspartate Aminotransferases ; Bezafibrate* ; Clofibrate ; Diabetic Nephropathies ; Diuretics ; Fructose-Bisphosphate Aldolase ; Humans ; Incidence ; Lower Extremity ; Middle Aged ; Renal Dialysis ; Renal Insufficiency ; Rhabdomyolysis*

OBJECTIVE: The idiopathic inflammatory myopathies (IIMs) are chronic systemic connective tissue diseases. The muscle biopsy is a definitive diagnostic tool but blind biopsy sometimes produces to negative results. Magnetic resonance imaging (MRI) as a tool for early diagnosis, guidance for biopsy, assessing extent of lesions and monitoring therapy in IIMs has been reported. The aim of this study is to assess the association of thigh inflammation through MRI and biopsy specimens with clinical findings. METHODS: Sixty patients diagnosed with dermatomyositis (DM) or polymyositis (PM) from 2004 to 2011 in one center of rheumatology were enrolled. We reviewed clinical, laboratory, histopathologic and MRI of thigh data at initial diagnosis. The inflammation grades by MRI and histopathology of muscles were evaluated through 4-point scoring systems. RESULTS: The laboratory findings for aldolase and CK differed significantly between DM patients (68.3%) and PM patients (31.7%). Fasciitis was detected by MRI in 43.3% of patients, of whom 88.5% had DM (p<0.05). The fasciitis was also associated with myalgia (p<0.05). Almost all MRI findings were symmetric except for two patients. The mean of total signal intensity was higher in patients with decreased muscle power. The signal intensity of affected muscle was slightly associated with muscle enzymes and histopathologic grading. CONCLUSION: Fasciitis was observed more in DM patients. MRI findings were associated with muscle enzymes and histopathologic grading. Signal intensity on MRI may be useful for measurement of disease activity in acute IIMs. The noninvasive nature and high sensitivity of muscle inflammation suggest that MRI images should be considered prior to muscle biopsy and treatment of IIMs.
Biopsy ; Connective Tissue Diseases ; Dermatomyositis ; Early Diagnosis ; Fasciitis ; Fructose-Bisphosphate Aldolase ; Humans ; Inflammation ; Magnetic Resonance Imaging* ; Magnetics* ; Magnets* ; Muscles ; Myositis* ; Polymyositis ; Rheumatology ; Thigh