Genetic evidence for the common identity of glucose-6-phosphatase, pyrophosphate-glucose phosphotransferase, carbamyl phosphate-glucose phosphotransferase and inorganic pyrophosphatase

We demonstrate that glucose-6-phosphatase, pyrophosphate-glucose phosphotransferase, carbamyl phosphate-glucose phosphotransferase and inorganic pyrophosphatase activities are deficient in livers of patients with type I glycogen storage disease. This provides strong genetic evidence that these enzym...

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Bibliographic Details
Published inBiochimica et biophysica acta Vol. 496; no. 2; pp. 431 - 435
Main Authors Hefferan, Patrick M., Rodney Howell, R.
Format Journal Article
LanguageEnglish
Published Netherlands Elsevier B.V 28.02.1977
Subjects
Carbamyl Phosphate - metabolism
Diphosphates - metabolism
Glucose - metabolism
Glucose-6-Phosphatase - metabolism
Glycogen Storage Disease Type I - metabolism
Humans
Liver - enzymology
Phosphotransferases - metabolism
Pyrophosphatases - metabolism
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Summary:We demonstrate that glucose-6-phosphatase, pyrophosphate-glucose phosphotransferase, carbamyl phosphate-glucose phosphotransferase and inorganic pyrophosphatase activities are deficient in livers of patients with type I glycogen storage disease. This provides strong genetic evidence that these enzymatic activities reside in a single protein or share a common polypeptide chain.
Bibliography:ObjectType-Article-2
SourceType-Scholarly Journals-1
ObjectType-Feature-1
content type line 23
ISSN:0304-4165
0006-3002
1872-8006
DOI:10.1016/0304-4165(77)90325-7