branching enzyme การใช้

• GBED is caused by an autosomal recessive mutation to the GBE1 gene, which leads glycogen branching enzyme activity that is reduced to absent.
• Horses that are heterozygous for the GBED allele have been shown to have glycogen branching enzyme activity levels that were half that of an unaffected horse.
• The branching enzyme can act upon only a branch having at least 11 residues, and the enzyme may transfer to the same glucose chain or adjacent glucose chains.
• There are currently 3 subcategories of glycogen storage diseases in horses : Type 1 Polysaccharide Storage Myopathy, Glycogen Branching Enzyme Deficiency ( GBED ), and Type 2 Polysaccharide Storage Myopathy.
• Specifically in this breed, complex rearrangements of glycogen branching enzyme ( GBE1 ) can cause a perinatal hypoglycaemic collapse and a late-juvenile-onset neuromuscular degeneration in glycogen storage disease type IV.
• The glycogen branching enzyme catalyzes the transfer of a terminal fragment of six or seven glucose residues from a nonreducing end to the C-6 hydroxyl group of a glucose residue deeper into the interior of the glycogen molecule.
• Glycoside hydrolase family 57 CAZY GH _ 57 comprises enzymes with several known activities; alpha-amylase ( ), 4-alpha-glucanotransferase ( ), ?-galactosidase ( ); amylopullulanase ( ); branching enzyme ( ).
• Due to the heavy influx of American Quarter Horse breeding, some Paints may also carry genetic disorders such as Hyperkalemic Periodic Paralysis ( HYPP ), Hereditary Equine Regional Dermal Asthenia ( HERDA ), Equine polysaccharide storage myopathy ( called PSSM-polysaccharide storage myopathy-in Paints, Quarter Horses and Appaloosas ), Malignant hyperthermia ( MH ) and Glycogen Branching Enzyme Deficiency ( GBED ).