title: Partial correction of an inherited biochemical defect of skeletal muscle by grafts of normal muscle precursor cells.
abstract: We have attempted to use allografts of normal muscle precursor cells (mpc) to insert donor nuclei, containing a normal genome, into growing or regenerating skeletal muscle fibres of mice with an inherited deficiency of the enzyme phosphorylase kinase (PhK). Analysis of the glucose-6-phosphate isomerase (GPI) isoenzymes of treated muscles showed that myonuclei of donor origin became incorporated into host muscle fibres in 8 of 9 regenerating autografts, but PhK activity was found only in the 3 grafts into which the largest numbers (1-3 x 10(6)) of mpc had been implanted. Following injection of normal mpc into growing PhK-deficient skeletal muscle, mosaic fibres containing myonuclei of donor origin were detected in only 11 of 192 muscles examined from 64 mice, but, of these 11 muscles, 5 contained PhK activity detectable by two separate assays in a further 4 muscles activity was detected by one or other assay.
authors.affiliation: Department of Histopathology, Charing Cross and Westminster Medical School, London, U.K.
authors.forename: J E
authors.initials: JE
authors.lastname: Morgan
authors.forename: D J
authors.initials: DJ
authors.lastname: Watt
authors.forename: J C
authors.initials: JC
authors.lastname: Sloper
authors.forename: T A
authors.initials: TA
authors.lastname: Partridge
mesh_terms: D000818:Animals; D000831:Animals, Newborn; D003433:Crosses, Genetic; D005260:Female; D005956:Glucose-6-Phosphate Isomerase / Q000378:metabolism; D008297:Male; D051379:Mice; D008810:Mice, Inbred C57BL; D008808:Mice, Inbred CBA; D008817:Mice, Mutant Strains; D008819:Mice, Nude; D009132:Muscles / Q000201:enzymology / Q000637:transplantation*; D010764:Phosphorylase Kinase / Q000172:deficiency* / Q000235:genetics / Q000378:metabolism; D012038:Regeneration
chemical_list: D010764:Phosphorylase Kinase; D005956:Glucose-6-Phosphate Isomerase