Pompe's disease - RePub - Erasmus Universiteit Rotterdam

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Cardiac remodeling and contractile function in KO-mice Fig. 3. Relation between hemodynamic variables under control conditions (open symbols) and during intravenous dobutamine infusion (solid symbols) LV to body weight ratio (LVW/BW), in ketamine-xylazine anesthetized mice. Upward triangles represent 20-24 months old C57Bl/6 WT mice; downward triangles represent 20-24 months old C57Bl/6 KO mice. Squares represent 10-14 months old FVB WT mice; diamonds represent 10-14 months old FVB KO mice. Dobutamine Intravenous infusion of dobutamine increased heart rate, diastolic arterial pressure, LV systolic pressure, LV dP/dt max and LV dP/dt min , and decreased τ, but had no effect on LV end-diastolic pressure in both WT and KO mice (Table 3). There were no differences in the responses to dobutamine in KO and WT mice. During dobutamine infusion, the inverse correlation between LVW/BW and LV dP/dt max was maintained (r 2 =0.62), but the inverse correlation between LVW/BW and diastolic arterial pressure was not signifi cant (Fig. 3), indicating that the lower LV dP/dt max during dobutamine was principally the result of a lower global LV contractility. During dobutamine infusion, the relation between LVW/BW and LV dP/dt min was maintained but τ was no longer different in KO and WT mice. The doses of dobutamine needed to produce maximal increases in LV dP/dt max in KO and WT mice were 9±2 µg/kg/min and 8±2 µg/kg/ min, respectively. 39
Chapter 2 Hemodynamics during isofl urane anesthesia Baseline hemodynamics Heart rate and LV dP/dt max were signifi cantly higher during isofl urane anesthesia (Table 4) than during ketamine-xylazine anesthesia (Table 3). Despite these differences in hemodynamics produced by the two anesthesia regimen, heart rate (471±28 versus 581±23 beats/min) and LV dP/dt max (5400±670 versus 8250±710 mmHg/s) were again lower in KO than in WT mice (both P
Page 2 and 3: Pompe’s disease The mouse as mode
Page 4 and 5: Pompe’s disease The mouse as mode
Page 6: Contents Chapter 1 Introduction 11
Page 10: 1Introduction
Page 13 and 14: Chapter 1 In lysosomal storage diso
Page 15 and 16: Chapter 1 Network are associated wi
Page 17 and 18: Chapter 1 the indirect approach can
Page 19 and 20: Chapter 1 Diagnosis Clinical Diagno
Page 21 and 22: Chapter 1 Fig. 3. Targeting vectors
Page 23 and 24: Chapter 1 muscles in approximately
Page 25 and 26: Chapter 1 1.7 Scope The studies des
Page 27 and 28: Chapter 1 47. Rudolph, C., et al.,
Page 29 and 30: Chapter 1 112. Dorling, P.R., J.M.
Page 32: 2 Cardiac Remodeling and Contractil
Page 35 and 36: Chapter 2 Experimental groups The a
Page 37 and 38: Chapter 2 Macroscopy The wet weight
Page 39: Chapter 2 Pooling of all individual
Page 43 and 44: Chapter 2 Discussion The major fi n
Page 45 and 46: Chapter 2 References 1. Bharati S,
Page 48: 3 Long Term Intravenous Treatment o
Page 51 and 52: Chapter 3 gene, we have focused on
Page 53 and 54: Chapter 3 pattern of alpha-glucosid
Page 55 and 56: Chapter 3 Alpha-glucosidase activit
Page 57 and 58: Chapter 3 and 4, respectively. The
Page 59 and 60: Chapter 3 For patient 1 the mental
Page 61 and 62: Chapter 3 R.M. Koppenol, T. de Vrie
Page 64: 4 Morphological Changes in Muscle T
Page 67 and 68: Chapter 4 Therapeutic regimen Pharm
Page 69 and 70: Chapter 4 Fig. 2. Pre treatment var
Page 71 and 72: Chapter 4 Fig. 4. Effect of ERT on
Page 73 and 74: Chapter 4 Table 2. Patients scores
Page 75 and 76: Chapter 4 enzyme. Moreover, when th
Page 78: 5Hearing loss in Infantile Pompe’
Page 81 and 82: Chapter 5 Methods Patients Seven pa
Page 83 and 84: Chapter 5 revealed a hearing loss o
Page 85 and 86: Chapter 5 Fig. 3. Threshold levels
Page 87 and 88: Chapter 5 Discussion Hearing loss i
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6 Both Low and High Uptake Forms of
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Chapter 6 healthy volunteers, we st
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Chapter 6 was extracted from human
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Chapter 6 from bovine testes (bAGLU
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Chapter 6 Fig. 5. Immunocytochemica
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Chapter 6 glucuronidase, and is obv
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Chapter 6 van Deel MSc for technica
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7Discussion
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Chapter 7 Cardiac function The mous
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Chapter 7 After the fi rst three mo
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Chapter 7 in 2004. This is 40 years
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Chapter 7 the skeletal muscle. The
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Chapter 7 46. Baudhuin, P., H.G. He
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age are ventilator dependent and wh
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om de morfologie van de spieren te
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naar de ziekte van Pompe bij kinder
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Publications Cardiac remodeling and
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Dankwoord Of ‘Mice and men’ zou
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Pompe's disease - RePub - Erasmus Universiteit Rotterdam

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