Letter: Preliminary x-ray data from well-ordered crystals of a human immunoglobulin G molecule.
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Biomedical subjects
Publications and source records attributed to W Palm.
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A variant form of maple syrup urine disease (grade II) in a twelve year old boy is reported. The clinical picture was characterized by seizure-like episodes of confusion and intermittent ataxia. The diagnosis was made by showing an increased excretion of branched-chain alpha-hydroxy acids as well as evaluated plasma concentrations of the branched-chain aminoacids and alpha-ketoacids. There was a decrease of leucine degradation in cultured fibroblasts to 5 to 6% of normal. The treatment with thiamine-hydrochlorid remained without any clinical or biochemical effect in our patient. Further neurologic symptoms during acute episodes of vomiting could be avoided by dietary protein restriction and early parenteral glucose supplementation.
The skin of Helix pomatia was investigated by biochemical methods and electronmicroscope. Biochemical investigations showed that Helix pomatia collagen represents a methionine-lacking collagen resistant to CNBr-cleavage. Electron microscopic studies showed a cross striation pattern of 53--57 nm.