Biomaterial Database

Lymphocyte phosphorylase kinase activities in the sex-linked form of liver phosphorylase kinase deficiency. 1985

K Goji, and Y Morishita, and S Kodama, and T Takahashi, and T Matsuo

Lymphocyte phosphorylase kinase activities were measured in normal controls and in patients with the sex-linked form of liver phosphorylase kinase deficiency. The reaction due to phosphorylase kinase activity in normal lymphocytes (2.7 X 10(6) in the reaction tube) was found to be linear within 20-60 min at 30 degrees C. The reaction was directly proportional to the concentration of lymphocytes within 1.5 X 10(6)-9.0 X 10(6), at 30 degrees C for 60 min. The phosphorylase kinase activity in normal lymphocytes, which were pre-incubated at 50 degrees C or 95 degrees C for 1 min, decreased to 60% at 50 degrees C and 10% at 95 degrees C of that after pre-incubation at 0 degree C for 1 min. The activity of normal controls was 125 +/- 23.5 U/10(10) lymphocytes. Those of the patients with liver phosphorylase kinase deficiency due to the sex-linked form were 43.5 U in case 1, 54.5 U in case 2, and 51.3 U in case 3, respectively and those of the mothers were within the normal range. These results suggest that phosphorylase kinase in lymphocytes might be form intermediate between liver and muscle phosphorylase kinase.

UI	MeSH Term	Description	Entries
D008099	Liver	A large lobed glandular organ in the abdomen of vertebrates that is responsible for detoxification, metabolism, synthesis and storage of various substances.	Livers
D008107	Liver Diseases	Pathological processes of the LIVER.	Liver Dysfunction,Disease, Liver,Diseases, Liver,Dysfunction, Liver,Dysfunctions, Liver,Liver Disease,Liver Dysfunctions
D008214	Lymphocytes	White blood cells formed in the body's lymphoid tissue. The nucleus is round or ovoid with coarse, irregularly clumped chromatin while the cytoplasm is typically pale blue with azurophilic (if any) granules. Most lymphocytes can be classified as either T or B (with subpopulations of each), or NATURAL KILLER CELLS.	Lymphoid Cells,Cell, Lymphoid,Cells, Lymphoid,Lymphocyte,Lymphoid Cell
D008297	Male		Males
D010375	Pedigree	The record of descent or ancestry, particularly of a particular condition or trait, indicating individual family members, their relationships, and their status with respect to the trait or condition.	Family Tree,Genealogical Tree,Genealogic Tree,Genetic Identity,Identity, Genetic,Family Trees,Genealogic Trees,Genealogical Trees,Genetic Identities,Identities, Genetic,Tree, Family,Tree, Genealogic,Tree, Genealogical,Trees, Family,Trees, Genealogic,Trees, Genealogical
D010764	Phosphorylase Kinase	An enzyme that catalyzes the conversion of ATP and PHOSPHORYLASE B to ADP and PHOSPHORYLASE A.	Glycogen Phosphorylase Kinase,Phosphorylase b Kinase,Kinase, Glycogen Phosphorylase,Kinase, Phosphorylase,Kinase, Phosphorylase b,Phosphorylase Kinase, Glycogen,b Kinase, Phosphorylase
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man