Analysis of Creatine Kinase Activity in 504 Patients with Proximal Spinal Muscular Atrophy Types I–III from the Point of View of Progression and Severity

1998 ◽  
Vol 39 (3) ◽  
pp. 154-162 ◽  
Author(s):  
Sabine Rudnik-Schöneborn ◽  
Susanne Lützenrath ◽  
Janina Borkowska ◽  
Anna Karwanska ◽  
Irena Hausmanowa-Petrusewicz ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Spinal Muscular Atrophy ◽  
Kinase Activity ◽  
Muscular Atrophy ◽  
Creatine Kinase Activity ◽  
Point Of View ◽  
Proximal Spinal Muscular Atrophy

scholarly journals Serum creatine kinase and creatinine in adult spinal muscular atrophy under nusinersen treatment

2021 ◽  
Author(s):  
Maren Freigang ◽  
Claudia D. Wurster ◽  
Tim Hagenacker ◽  
Benjamin Stolte ◽  
Markus Weiler ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Spinal Muscular Atrophy ◽  
Muscular Atrophy ◽  
Serum Creatine Kinase

Distribution of serum creatine kinase activity in young healthy persons

1999 ◽  
Vol 279 (1-2) ◽  
pp. 107-115 ◽  
Author(s):  
Eli I. Lev ◽  
Ilan Tur-Kaspa ◽  
Isaac Ashkenazy ◽  
Anat Reiner ◽  
David Faraggi ◽  
...  
Keyword(s):  
Creatine Kinase ◽  
Kinase Activity ◽  
Creatine Kinase Activity ◽  
Serum Creatine Kinase ◽  
Healthy Persons

SETX gene mutation in a family diagnosed autosomal dominant proximal spinal muscular atrophy

2012 ◽  
Vol 22 (3) ◽  
pp. 258-262 ◽  
Author(s):  
Sabine Rudnik-Schöneborn ◽  
Larissa Arning ◽  
Jörg T. Epplen ◽  
Klaus Zerres
Keyword(s):  
Spinal Muscular Atrophy ◽  
Gene Mutation ◽  
Autosomal Dominant ◽  
Muscular Atrophy ◽  
Proximal Spinal Muscular Atrophy

scholarly journals Ability of adenovirus 5 E1A proteins to suppress differentiation of BC3H1 myoblasts correlates with their binding to a 300 kDa cellular protein.

1992 ◽  
Vol 3 (10) ◽  
pp. 1107-1115 ◽  
Author(s):  
J S Mymryk ◽  
R W Lee ◽  
S T Bayley
Keyword(s):  
Creatine Kinase ◽  
Kinase Activity ◽  
Cellular Protein ◽  
Creatine Kinase Activity ◽  
Cellular Proteins ◽  
Deletion Mutants ◽  
Transcriptional Enhancers ◽  
Actin Mrna ◽  
Alpha Actin ◽  
Adenovirus 5

We have used deletion mutants to define the regions in Ad5 E1A proteins necessary to suppress differentiation of mouse BC3H1 myoblasts. We examined the differentiation of cells infected at a low multiplicity with viruses containing the E1A deletions and constructed so as to produce only the smaller of the two major E1A proteins. Only four of the mutant viruses containing deletions within the N-terminal 69 residues failed to suppress differentiation as judged by changes in morphology and in levels of muscle-specific alpha-actin mRNA and creatine kinase activity. The results were confirmed by analyses of lines of cells stably transfected with representative E1A mutants. The mouse cellular proteins to which mutant E1A proteins bound were identified by immunoprecipitating E1A proteins specifically from infected BC3H1 cells and by analyzing the precipitates on denaturing gels. Bands of proteins of 300, 130, 107, 105 (the retinoblastoma product), and 60 kDa (cyclin A) were distinguished. Failure to suppress differentiation correlated with loss of binding to the 300-kDa protein but not to any of the others. The regions of E1A defined in this way have been shown to be required for several other activities, including enhancer repression and transformation. One function of the 300-kDa protein appears to be to facilitate the action of transcriptional enhancers of differentiation-specific genes.

Skeletal muscle collagen content in humans after high-force eccentric contractions

2004 ◽  
Vol 97 (1) ◽  
pp. 197-203 ◽  
Author(s):  
Abigail L. Mackey ◽  
Alan E. Donnelly ◽  
Taina Turpeenniemi-Hujanen ◽  
Helen P. Roper
Keyword(s):  
Creatine Kinase ◽  
Kinase Activity ◽  
Staining Intensity ◽  
Creatine Kinase Activity ◽  
Serum Creatine Kinase ◽  
Muscle Contractions ◽  
Tissue Inhibitors Of Metalloproteinases ◽  
Eccentric Contractions ◽  
Knee Extensors ◽  
Type Iv

The purpose of this study was to investigate the effects of high-force eccentric muscle contractions on collagen remodeling and on circulating levels of matrix metalloproteinases (MMP) and tissue inhibitors of metalloproteinases (TIMP) in humans. Nine volunteers [5 men and 4 women, mean age 23 (SD 4) yr] each performed a bout of 100 maximum voluntary eccentric contractions of the knee extensors. Muscle biopsies were taken before exercise and on days 4 and 22 afterward. Image analysis of stained tissue sections was used to quantify endomysial collagen staining intensity. Maximum voluntary contractile isometric force was recorded preexercise and on days 1, 2, 3, 4, 8, 11, and 14 postexercise. Venipuncture blood samples were also drawn on these days for measurement of serum creatine kinase activity and concentrations of MMP-9, TIMP-1, TIMP-2, and the MMP-2/TIMP-2 complex. Maximum voluntary contractile force declined by 39 ± 23% (mean ± SD) on day 2 postexercise and recovered thereafter. Serum creatine kinase activity peaked on day 4 postexercise ( P < 0.01). Collagen type IV staining intensity increased significantly on day 22 postexercise to 126 ± 29% (mean ± SD) of preexercise values ( P < 0.05). Serum MMP-9 levels increased on day 8 postexercise ( P < 0.01), and serum TIMP-1 was also significantly elevated on days 1, 2, 3, 4, and 14 postexercise ( P < 0.05). These results suggest that a single bout of eccentric muscle contractions results in remodeling of endomysial type IV collagen, possibly via the MMP pathway.

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