Gsd Type Vi Liver Phosphorylase Hers Disease

The enzyme deficient in type VI GSD is liver-specific phosphorylase. It is relatively rare and a benign form of GSD with no heart and skeletal muscle involvement. Most patients with GSD VI disease present with hepatomegaly and growth retardation. Hypoglycemia, hyperlipidemia, and hyperketosis, if present, are usually mild. The hepatomegaly improves and disappears around puberty. Diagnosis rests on enzyme analysis of the liver biopsy. GSD VI is autosomal recessive. Liver phosphorylase gene (PYGL) has been mapped to chromosome 14q21 and has 20 exons.[23] A splice site mutation in intron 13 has been identified in Mennonite population.

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Getting Started With Dumbbells

Getting Started With Dumbbells

The use of dumbbells gives you a much more comprehensive strengthening effect because the workout engages your stabilizer muscles, in addition to the muscle you may be pin-pointing. Without all of the belts and artificial stabilizers of a machine, you also engage your core muscles, which are your body's natural stabilizers.

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