Isoenzyme distribution of creatine kinase and lactate dehydrogenase in serum and skeletal muscle in Duchenne muscular dystrophy, collagen disease, and other muscular disorders.

1978 ◽  
Vol 24 (11) ◽  
pp. 1985-1989 ◽  
Author(s):  
W G Yasmineh ◽  
G A Ibrahim ◽  
M Abbasnezhad ◽  
E A Awad
Keyword(s):  
Skeletal Muscle ◽  
Creatine Kinase ◽  
Lactate Dehydrogenase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Serum Creatine Kinase ◽  
Collagen Disease ◽  
Total Serum ◽  
Control Group ◽  
The Mean

Abstract We determined the total activity and isoenzyme distribution of lactate dehydrogenase and creatine kinase in serum and biopsy specimens from skeletal muscle of nine normal individuals and nine patients with Duchenne muscular dystrophy (I), five with collagen disease (II), and four with non-progressive unclassified myopathy (III). Mean total serum creatine kinase in patients with Duchenne muscular dystrophy (867 U/liter, SD = 197) was 31-fold that in the control group (28 U/liter, SD = 14). There was also a small (3.3-fold) increase in the mean total serum creatine kinase of patients with III, but none in the serum from patients with II. Changes in the creatine kinase isoenzyme distribution of skeletal muscle were primarily in the MB isoenzyme. The mean percentage of creatine kinase-MB activity in muscle from patients with I (2.81, SD = 1.15) and patients with III (1.69, SD = 1.07) significantly (P less than 0.005) exceeded that of the control group (0.43, SD = 0.18). Muscle from patients with II showed little change. The most striking changes in lactate dehydrogenase were also observed in patients with I, in whom the mean total serum activity (356 U/liter, SD = 115) was 3.4-fold that of serum from the control group (105 U/liter, SD = 19). Skeletal muscle from these patients also showed a significant decrease in mean percent isoenzyme 5 activity (from 50 to 23) and an increase in that of isoenzymes 1 and 2 (from 1 to 9 and 8 to 20, respectively). These changes in the distribution of these two sets of isoenzymes in muscle were reflected in the serum.

scholarly journals Characterization of a Blood Spot Creatine Kinase Skeletal Muscle Isoform Immunoassay for High-Throughput Newborn Screening of Duchenne Muscular Dystrophy

2017 ◽  
Vol 63 (4) ◽  
pp. 908-914 ◽  
Author(s):  
Stuart J Moat ◽  
Teemu Korpimäki ◽  
Petra Furu ◽  
Harri Hakala ◽  
Hanna Polari ◽  
...  
Keyword(s):  
Skeletal Muscle ◽  
Creatine Kinase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Newborn Screening ◽  
High Throughput ◽  
Screening Programs ◽  
Blood Spots ◽  
The Mean ◽  
Blood Spot

Abstract BACKGROUND Duchenne muscular dystrophy (DMD) is a progressive, lethal X-linked neuromuscular disorder with an average worldwide incidence of 1:5000. Blood spot creatine kinase (CK) enzyme assays previously used in newborn screening programs for DMD are nonspecific because measured CK enzyme activity is attributable to 3 isoenzyme forms of CK (CK-MM, CK-MB, and CK-BB) and it is the CK-MM isoform that is found predominantly in skeletal muscle. CK-MM is increased in boys with DMD owing to muscle damage. We describe a sensitive and specific automated immunoassay for CK-MM to screen for DMD in blood spots. METHODS The prototype assay was developed on the PerkinElmer GSP® analyzer to enable high-throughput screening. CK-MM was assayed using a solid phase, 2-site immunofluorometric system. Purified human CK-MM was used to create calibrators and controls. RESULTS The limit of blank (LOB), detection (LOD), and quantification (LOQ) values were <1, 3, and 8 ng/mL, respectively. The analytical measurement range was 4–8840 ng/mL. Interassay (n = 40) imprecision was <7% across the analytical range. Cross-reactivity was <5% for CK-MB and 0% for CK-BB. The mean recovery of CK-MM was 101% (range 87%–111%). Blood spots from newborn infants (n = 277) had a mean CK-MM concentration of 155 ng/mL and a 99th centile of 563 ng/mL. The mean blood spot CK-MM concentration from 10 cases of DMD was 5458 ng/mL (range 1217–9917 ng/mL). CONCLUSIONS CK-MM can be reliably quantified in blood spots. The development of this CK-MM assay on a commercial immunoassay analyzer would enable standardized and high-throughput newborn blood spot screening of DMD.

Dilution Effect on Serum Creatine Kinase in Carriers of Duchenne Muscular Dystrophy

1979 ◽  
Vol 16 (1-6) ◽  
pp. 54-55 ◽  
Author(s):  
A. C. Simpson ◽  
D. Holmes ◽  
R. J. T. Pennington
Keyword(s):  
Creatine Kinase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Dilution Effect ◽  
Serum Creatine Kinase ◽  
Normal Female ◽  
Female Subjects

Dilution of serum before assay consistently increased the activity of serum creatine kinase both in normal female subjects and in carriers of Duchenne muscular dystrophy.

scholarly journals Effect of exercise on serum creatine kinase in carriers of Duchenne muscular dystrophy.

1982 ◽  
Vol 19 (1) ◽  
pp. 4-7 ◽  
Author(s):  
R F Gaines ◽  
S M Pueschel ◽  
E A Sassaman ◽  
J L Driscoll
Keyword(s):  
Creatine Kinase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Serum Creatine Kinase

Glutathione depletion during experimental damage to rat skeletal muscle and its relevance to Duchenne muscular dystrophy

1991 ◽  
Vol 80 (6) ◽  
pp. 559-564 ◽  
Author(s):  
M. J. Jackson ◽  
M. H. Brooke ◽  
K. Kaiser ◽  
R. H. T. Edwards
Keyword(s):  
Skeletal Muscle ◽  
Creatine Kinase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Time Course ◽  
Contractile Activity ◽  
Calcium Ionophore ◽  
Glutathione Depletion ◽  
Ionophore A23187 ◽  
Enzyme Release

1. The release of glutathione has been studied in comparison with the release of creatine kinase from isolated rat soleus muscles subjected to certain forms of experimental damage. 2. Excessive electrically stimulated contractile activity or treatment of muscles with the mitochondrial inhibitor, 2,4-dinitrophenol, induced a substantial release of both creatine kinase and glutathione and a reduction in the total glutathione content of the muscle. The time course of this release and depletion indicates that the efflux of the two molecules is not directly related and that a reduction in muscle glutathione content does not occur before cytosolic enzyme release. 3. 2,4-Dinitrophenol-stimulated release of creatine kinase was significantly reduced by the omission of external calcium from the incubation media, but glutathione release and depletion was relatively unaffected by this. Deliberate elevation of the muscle intracellular calcium content with the calcium ionophore, A23187, induced a substantial loss of creatine kinase, but had no significant effect on the release of glutathione. 4. Muscle biopsies from patients with Duchenne muscular dystrophy were found to have an elevated content of glutathione and an equivalent protein-thiol content compared with control subjects. 5. We conclude that, although release of glutathione from skeletal muscle occurs after excessive contractile activity or inhibition of mitochondrial metabolism, this is not a key step in the damaging processes leading to cytosolic enzyme release, neither is it relevant to the ongoing damage to skeletal muscle which occurs in patients with Duchenne muscular dystrophy.

SERUM CREATINE KINASE ACTIVITY AND CARRIER STATUS FOR DUCHENNE MUSCULAR DYSTROPHY

The Lancet ◽  
1979 ◽  
Vol 314 (8156-8157) ◽  
pp. 1370 ◽  
Author(s):  
D.G. Bullock ◽  
F.M. Mcsweeney ◽  
T.P. Whitehead ◽  
J.H. Edwards
Keyword(s):  
Creatine Kinase ◽  
Duchenne Muscular Dystrophy ◽  
Muscular Dystrophy ◽  
Kinase Activity ◽  
Creatine Kinase Activity ◽  
Serum Creatine Kinase ◽  
Carrier Status
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