65 research outputs found
Glucose-metabolism in a Child With "3-hydroxy-3-methylglutaryl-coenzyme a Lyase Deficiency
No full textPeroxisomal matrix enzymes in Zellweger syndrome: activity and subcellular localization in liver
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Folate-responsive homocystinuria and megaloblastic anaemia in a female patient with functional methionine synthase deficiency (cblE disease)
No full textProperties of the Atpase Activity Associated with Peroxisome-Enriched Fractions From Rat-Liver - Comparison with Mitochondrial F1F0-Atpase
No full textHighly purified peroxisomal fractions from rat liver contain ATPase activity (18.8 +/- 0.1 nmol/min per mg, n = 6). This activity is about 2% of that found in purified mitochondrial fractions. Measurement of marker enzyme activities and immunoblotting of the peroxisomal fraction with an antiserum raised against the beta-subunit of mitochondrial ATPase indicates that the ATPase activity in the peroxisomal fractions can not be ascribed to contamination with mitochondria or other subcellular organelles. From the sensitivity of the ATPase present in the peroxisomal fraction towards a variety of ATPase inhibitors, we conclude that it displays both V-type and F-type features and is distinguishable from both the mitochondrial F1F0-ATPase and the lysosomal V-type ATPas
Folate-responsive homocystinuria and megaloblastic anaemia in a female patient with functional methionine synthase deficiency (cblE disease)
No full textClinical recognition of patients affected by a peroxisomal disorder: A retrospective study in 40 patients
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