Today, many microbial amylases are available commercially and they have almost completely replaced chemical hydrolysis in several industry processes. Amylases from microorganisms have a broad spectrum of industrial applications as they are more stable than amylases obtained from plants and animals. The objective of this work was to use potato baits in an Atlantic Forest remnant located in Ribeirão Preto, São Paulo, Brazil, in order to obtain amylase-producing fungi with potential for biotechnological application. In addition, the culture conditions for the fungal strain that presented higher production of glucoamylase were standardized using industrial wastes. For this, 6 PET bottles containing potatoes as baits were scattered at different points in an Atlantic forest remnant. After 6 days, the samples were collected, and the filamentous fungi were isolated in Petri dishes. Fungi screening was carried out in Khanna liquid medium with 1% starch Reagen®, at 30 °C, pH 6.0, under static conditions for 4 days. Proteins and glucoamylase activity were determined by Bradford and 3,5-dinitrosalicylic acid (DNS), respectively. Among all isolated fungi, A. carbonarius showed the highest glucoamylase production. Its best cultivation conditions were observed in Khanna medium, 4 days, at 30 °C, pH 6.0, under static condition with 0.1% yeast extract and 1% starch Reagen®. Wheat and brewing residues were also used as inducers for large quantities of glucoamylase production. A. carbonarius showed to be a good alternative for the wheat and brewing waste destinations in order to obtain high added value products.
- MeSH
- Aspergillus enzymology isolation & purification MeSH
- Bioprospecting MeSH
- Glucan 1,4-alpha-Glucosidase metabolism MeSH
- Hydrolysis MeSH
- Forests MeSH
- Triticum metabolism MeSH
- Starch metabolism MeSH
- Tropical Climate MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- Brazil MeSH
- MeSH
- Diet Therapy MeSH
- Child MeSH
- Glucan 1,4-alpha-Glucosidase blood deficiency MeSH
- Glycogen Storage Disease Type III * genetics physiopathology therapy MeSH
- Hepatomegaly enzymology pathology MeSH
- Hypoglycemia enzymology MeSH
- Humans MeSH
- Starch therapeutic use MeSH
- Check Tag
- Child MeSH
- Humans MeSH
- Male MeSH
- Publication type
- Case Reports MeSH
Pompeho nemoc (glykogenóza typ 2, deficit alfa glukosidázy) je autozomálně recesivní dědičné metabolické onemocnění, jehož podkladem je defekt lysozomální kyselé alfa glukosidázy vedoucí k hromadění-střádání lysozomálního glykogenu v buňkách a tkáních s následnou dysfunkcí především ve svalové tkáni srdce a kosterních svalech. Průběh je velmi variabilní: od těžkého rychle progredujícího postižení novorozenců (klasická infantilní forma) po postupné postižení s manifestací v dětství či pozdní dospělosti. Je-li alfa glukosidáza zcela nebo téměř nefunkční, vzniká tzv. klasická infantilní forma choroby. Potíže se rozvíjí v prvních měsících života a mají charakter neprospívání, svalové slabosti a potíží s dýcháním. Dochází k výraznému zvětšení srdce, většina dětí se před zavedením substituční terapie nedožila prvních narozenin. Je-li enzym štěpící glykogen alespoň částečně funkční, vzniká tzv. pozdní (juvenilní či adultní) forma onemocnění. Tato situace nastává, pokud vznikne závažná mutace jedné alely a lehčí na druhé. To vede k méně progresivnímu fenotypu. Rozpětí manifestace nemoci je u této formy od první do šesté věkové dekády. Hlavním projevem je svalová slabost, která postupuje a významně zasahuje dýchací svalstvo. U těchto nemocných nebývá srdce zvětšeno. Při podezření na PN máme tři diagnostické úrovně. První je skríningové vyšetření pomocí testu suché kapky (DBS, Dried Blood Spot). Potvrzení diagnózy se provádí vyšetřením aktivity GAA v leukocytech. DNA vyšetření je důležité pro stanovení korelace genotyp-fenotyp a detekci přenašečů v rodině.
Pompe disease (glycogen storage disease type 2, acid maltase deficiency) is inherited autosomal recessive metabolic disorder caused by deficiency of acid alpha-glucosidase and resulting in lysosomal glycogen storage in various tissues, mainly heart and skeletal muscle. Continuous spectrum of phenotypes from the rapidly progressive infantile form to the slowly progressive late onset form of the disease can be observed. Classical infantile form of the disease manifests soon after birth due to absent or nearly absent activity of the key enzyme. Typical manifestations include failure to thrive, muscle weakness, cardiomegaly, and respiratory failure. Before the era of substitution therapy, the majority of children died within the first year of life. Partial enzyme deficiency (severe mutation on one allele and milder on the second) leads to the less severe phenotype with manifestation in child- or adulthood. Time span is from the first to the sixth decade of life. Leading symptoms include slowly progressing limb girdle and trunk muscle weakness with significant involvement of respiratory muscles. There is no cardiomegaly. Suspicion of Pompe disease is confirmed in three steps. The first involves screening with the Dried Blood Spot test. Testing of the activity of alfa glucosidase in leukocytes is used to confirm the disease. Mutation analysis is important to assess the correlation between genotype and phenotype and to identify familial carriers. Key words: Pompe disease – alpha glucosidase – lysosomal storage diseases – limb-girdle muscle weakness The author declares he has no potential conflicts of interest concerning drugs, products, or services used in the study. The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.
- Keywords
- test metodou suché kapky,
- MeSH
- alpha-Glucosidases therapeutic use MeSH
- Biopsy MeSH
- Diagnosis, Differential MeSH
- Adult MeSH
- Electromyography MeSH
- Genetic Testing MeSH
- Glucan 1,4-alpha-Glucosidase genetics deficiency MeSH
- Glycogen Storage Disease Type II * diagnosis epidemiology etiology physiopathology therapy MeSH
- Muscle, Skeletal physiopathology pathology MeSH
- Creatine Kinase blood MeSH
- Humans MeSH
- Mutation genetics MeSH
- Muscular Dystrophies, Limb-Girdle diagnosis MeSH
- Prognosis MeSH
- Disease Progression MeSH
- Respiratory Insufficiency * diagnosis complications MeSH
- Muscle Weakness * MeSH
- Rare Diseases MeSH
- Check Tag
- Adult MeSH
- Humans MeSH
- MeSH
- Amino Acid Motifs MeSH
- Aspergillus niger enzymology genetics MeSH
- Research Support as Topic MeSH
- Glucan 1,4-alpha-Glucosidase genetics chemistry metabolism MeSH
- Glucosyltransferases genetics chemistry metabolism MeSH
- Conserved Sequence MeSH
- Humans MeSH
- Muscle Proteins genetics chemistry MeSH
- Check Tag
- Humans MeSH
- Publication type
- Comparative Study MeSH
- MeSH
- alpha-Glucosidases diagnostic use MeSH
- beta-Galactosidase diagnostic use MeSH
- Chromatography, Affinity * methods utilization MeSH
- Electrophoresis, Polyacrylamide Gel methods utilization MeSH
- Glucan 1,4-alpha-Glucosidase diagnostic use MeSH
- Glycoside Hydrolases * isolation & purification metabolism MeSH
- Rats MeSH
- Lactase diagnostic use MeSH
- Sepharose diagnostic use isolation & purification MeSH
- Statistics as Topic MeSH
- Intestinal Mucosa enzymology MeSH
- Intestine, Small * enzymology metabolism MeSH
- Thioglucosides diagnostic use MeSH
- Animals MeSH
- Check Tag
- Rats MeSH
- Animals MeSH
