Biomaterial Database

Myotonic muscular dystrophy. Calcium-dependent phosphatidate metabolism in the erythrocyte membrane. 1982

L H Yamaoka, and J M Vance, and A D Roses

It has been suggested that the erythrocytes of myotonic dystrophy (MyD) patients have a decreased calcium-stimulated phosphatidic acid (PA) accumulation. This could be the result of a defect in the calcium-stimulated hydrolysis of the polyphosphoinositides (calcium-dependent phosphodiesterase) or in the subsequent formation of PA from its precursors (diacylglycerol kinase). In vitro assays were established for both enzymes in erythrocyte membranes. Calcium-dependent phosphodiesterase activity was assayed with both endogenous 32P-labeled erythrocyte diphosphoinositide and triphosphoinositide and with the same phospholipids isolated from rat brain. No significant differences in activity were found between MyD patients and normal controls with either method of substrate preparation. No difference in diglyceride kinase activity was found between ghosts prepared from MyD patients and normal controls. Thus, there were no differences in either of the membrane-associated enzymes of phosphatidic acid metabolism.

UI	MeSH Term	Description	Entries
D009136	Muscular Dystrophies	A heterogeneous group of inherited MYOPATHIES, characterized by wasting and weakness of the SKELETAL MUSCLE. They are categorized by the sites of MUSCLE WEAKNESS; AGE OF ONSET; and INHERITANCE PATTERNS.	Muscular Dystrophy,Myodystrophica,Myodystrophy,Dystrophies, Muscular,Dystrophy, Muscular,Myodystrophicas,Myodystrophies
D010712	Phosphatidic Acids	Fatty acid derivatives of glycerophosphates. They are composed of glycerol bound in ester linkage with 1 mole of phosphoric acid at the terminal 3-hydroxyl group and with 2 moles of fatty acids at the other two hydroxyl groups.	Ammonium Phosphatidate,Diacylglycerophosphates,Phosphatidic Acid,Acid, Phosphatidic,Acids, Phosphatidic,Phosphatidate, Ammonium
D010727	Phosphoric Diester Hydrolases	A class of enzymes that catalyze the hydrolysis of one of the two ester bonds in a phosphodiester compound. EC 3.1.4.	Phosphodiesterase,Phosphodiesterases,Hydrolases, Phosphoric Diester
D010770	Phosphotransferases	A rather large group of enzymes comprising not only those transferring phosphate but also diphosphate, nucleotidyl residues, and others. These have also been subdivided according to the acceptor group. (From Enzyme Nomenclature, 1992) EC 2.7.	Kinases,Phosphotransferase,Phosphotransferases, ATP,Transphosphorylase,Transphosphorylases,Kinase,ATP Phosphotransferases
D002118	Calcium	A basic element found in nearly all tissues. It is a member of the alkaline earth family of metals with the atomic symbol Ca, atomic number 20, and atomic weight 40. Calcium is the most abundant mineral in the body and combines with phosphorus to form calcium phosphate in the bones and teeth. It is essential for the normal functioning of nerves and muscles and plays a role in blood coagulation (as factor IV) and in many enzymatic processes.	Coagulation Factor IV,Factor IV,Blood Coagulation Factor IV,Calcium-40,Calcium 40,Factor IV, Coagulation
D004910	Erythrocyte Membrane	The semi-permeable outer structure of a red blood cell. It is known as a red cell 'ghost' after HEMOLYSIS.	Erythrocyte Ghost,Red Cell Cytoskeleton,Red Cell Ghost,Erythrocyte Cytoskeleton,Cytoskeleton, Erythrocyte,Cytoskeleton, Red Cell,Erythrocyte Cytoskeletons,Erythrocyte Ghosts,Erythrocyte Membranes,Ghost, Erythrocyte,Ghost, Red Cell,Membrane, Erythrocyte,Red Cell Cytoskeletons,Red Cell Ghosts
D004912	Erythrocytes	Red blood cells. Mature erythrocytes are non-nucleated, biconcave disks containing HEMOGLOBIN whose function is to transport OXYGEN.	Blood Cells, Red,Blood Corpuscles, Red,Red Blood Cells,Red Blood Corpuscles,Blood Cell, Red,Blood Corpuscle, Red,Erythrocyte,Red Blood Cell,Red Blood Corpuscle
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D054801	Phosphoinositide Phospholipase C	A type C phospholipase with specificity towards PHOSPHATIDYLINOSITOLS that contain INOSITOL 1,4,5-TRISPHOSPHATE. Many of the enzymes listed under this classification are involved in intracellular signaling.	1-Phosphatidyl-D-myo-Inositol-4,5-bisphosphate Inositoltrisphosphohydrolase,1-Phosphatidylinositol-4,5-Bisphosphate Phosphodiesterase,PIP2-PLC,PIP2-Phosphoinositidase C,PPI Phosphodiesterase,Phosphatidylinositide-4,5-Bisphosphate Phospholipase C,Phosphatidylinositol-4,5-Biphosphate-Phosphodiesterase,Phosphatidylinositol-Specific Phospholipase C,Phosphoinositide Selective Phospholipase C,Phosphoinositol Specific Phospholipase C,Polyphosphoinositide Phosphodiesterase,Polyphosphoinositide Phospholipase C,PtdIns(4,5)P2 Phosphodiesterase,Tri-phosphoinositide Phosphodiesterase,Triphosphoinositide Phosphodiesterase,1 Phosphatidyl D myo Inositol 4,5 bisphosphate Inositoltrisphosphohydrolase,1 Phosphatidylinositol 4,5 Bisphosphate Phosphodiesterase,Inositoltrisphosphohydrolase, 1-Phosphatidyl-D-myo-Inositol-4,5-bisphosphate,PIP2 Phosphoinositidase C,Phosphatidylinositide 4,5 Bisphosphate Phospholipase C,Phosphatidylinositol 4,5 Biphosphate Phosphodiesterase,Phosphatidylinositol Specific Phospholipase C,Phosphodiesterase, 1-Phosphatidylinositol-4,5-Bisphosphate,Phosphodiesterase, PPI,Phosphodiesterase, Polyphosphoinositide,Phosphodiesterase, Triphosphoinositide,Phospholipase C, Phosphatidylinositide-4,5-Bisphosphate,Phospholipase C, Phosphatidylinositol-Specific,Phospholipase C, Polyphosphoinositide,Tri phosphoinositide Phosphodiesterase
D019852	Diacylglycerol Kinase	An enzyme of the transferase class that uses ATP to catalyze the phosphorylation of diacylglycerol to a phosphatidate. EC 2.7.1.107.	DAG Kinase,Diacylglycerol Kinase alpha (DGKA),Diglyceride Kinase,1,2-Diacylglycerol Kinase,Arachidonoyl-Diacylglycerol Kinase,Ceramide-Diglyceride Kinase,DGK alpha,DGK beta,DGK delta,DGK gamma,DGK-IV,DGKdelta,1,2 Diacylglycerol Kinase,Arachidonoyl Diacylglycerol Kinase,Ceramide Diglyceride Kinase,Kinase, 1,2-Diacylglycerol,Kinase, Arachidonoyl-Diacylglycerol,Kinase, Ceramide-Diglyceride,Kinase, DAG,Kinase, Diacylglycerol,Kinase, Diglyceride,alpha, DGK,beta, DGK,delta, DGK,gamma, DGK