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

Vectors used to carry foreign genes play an important role in gene therapy, among which, the adeno-associated virus (AAV) has many advantages, such as nonpathogenicity, low immunogenicity, stable and long-term expression and multiple-tissue-type infection, etc. These advantages have made AAV one of the most potential vectors in gene therapy, and widely used in many clinical researches, for example, Parkinson's disease. This paper introduces the biological characteristics of AAV and the latest research progress of AAV carrying neurotrophic factor, dopamine synthesis related enzymes and glutamic acid decarboxylase gene in the gene therapy of Parkinson's disease.
Animals ; Aromatic-L-Amino-Acid Decarboxylases ; genetics ; Dependovirus ; genetics ; Gene Transfer Techniques ; Genetic Therapy ; Genetic Vectors ; Glial Cell Line-Derived Neurotrophic Factor ; genetics ; Glutamate Decarboxylase ; genetics ; Humans ; Nerve Growth Factors ; genetics ; Neurturin ; genetics ; Parkinson Disease ; therapy

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

A boy attended the hospital at the age of 1 month due to left hand tremor for 1 week. A blood test showed a reduction in serum uric acid and a cranial MRI showed encephalomalacia, atrophy, and cystic changes. The boy had microcephalus, unusual facial features (long face, long forehead, protruded forehead, long philtrum, low nasal bridge, facial swelling, and thick lower lip), hypertonia of lower extremities, and severe global developmental delay. Whole-exome sequencing performed for the boy detected a homozygous mutation, c.217C > T(p.R73W), in the
Carbon-Carbon Lyases ; China ; Humans ; Infant, Newborn ; Male ; Metal Metabolism, Inborn Errors ; Mutation ; Uric Acid