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lmnagt -/- lmnagt -/- mice lamin acad 9 mutation patient protein activity analysis mitochondrial tissue ficiency i activity muscle mouse +/- function post-natal ds i t lmnagt +/- development model flavin differentiation defect study wild-type acad 9 i i c lmnagt -/- mouse level heart expression hypertrophy ribo flavin wild-type effect phenotype cause assembly lmnagt +/- mice oxidative phosphorylation myocyte genotype broblast brain fi broblasts blood department adipose weight tibia length pp 15 subcutaneous adipose tissue ribo flavin treatment subunit region ventricle a-type genet lmnagt -/- vs nucleus lmnagt -/- scholte medium post-natal development acad 9. subcutaneou staining maastricht disease value control family institute identi fied exercise intolerance hydrogen bond structure
2 Most Recent Publications
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Post-natal myogenic and adipogenic developmental:Defects and metabolic impairment upon loss of a-type lamins
(Article)
Kubben, N. Voncken, J.W. Calis, C. Houten, S.M. Misteli, T. Pinto, Y.M. Koning, G.A. Weeghel, M. van Hoogenhof, M.M.G. van den Gijbels, M. Erk, A. van Schoonderwoerd, K. Bosch, B. van den Dahlmans, V. |
2011-07-18
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Riboflavin-responsive oxidative phosphorylation complex i deficiency caused by defective ACAD9: New function for an old gene
(Article)
Gerards, M. Bosch, B.J. van den Prokisch, H. Rötig, A. Coo, I.F.M. de Smeets, H.J. Danhauser, K. Serre, V. Weeghel, M. van Wanders, R.J.A. Nicolaes, G.A.F. Sluiter, W. Schoonderwoerd, K. Scholte, H.R. |
2011-01-01
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