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61.
Suppression of peroxisomal membrane protein defects by peroxisomal ATP binding cassette (ABC) proteins 总被引:8,自引:4,他引:4
Braiterman LT; Zheng S; Watkins PA; Geraghty MT; Johnson G; McGuinness MC; Moser AB; Smith KD 《Human molecular genetics》1998,7(2):239-247
X-Linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder
characterized by reduced peroxisomal very long chain fatty acid (VLCFA)
beta-oxidation. The X - ALD gene product (ALDP) is a peroxisomal
transmembrane protein with an ATP binding cassette (ABC). ALDP and three
other ABC proteins (PMP70, ALDR, P70R) localize to the peroxisomal
membrane. The function of this family of peroxisomal membrane proteins is
unknown. We used complementation studies to begin analysis of their role in
VLCFA beta-oxidation and on the peroxisomal membrane. Expression of either
ALDP or PMP70 restores VLCFA beta- oxidation in X-ALD fibroblasts,
indicating overlapping functions. Their expression also restores peroxisome
biogenesis in cells that are deficient in the peroxisomal membrane protein
Pex2p. Thus it is likely that complex protein interactions are involved in
the function and biogenesis of peroxisomal membranes that may contribute to
disease heterogeneity.
相似文献
62.
Steinsapir KD Goldberg RA Sinha S Hovda DA 《Journal of the peripheral nervous system : JPNS》2002,7(2):136-136
The excitability of human axons can be studied reliably using the technique of threshold tracking, which allows the strength of a test stimulus to be adjusted by computer to activate a defined fraction of the maximal nerve or muscle action potential. The stimulus current that just evokes the target response is considered the 'threshold' for that response. More useful than the resting threshold are other indices of axonal excitability derived from pairs of threshold measurements, such as refractoriness, supernormality, strength-duration time constant and 'threshold electrotonus' (i.e. the changes in threshold produced by long-lasting depolarizing or hyperpolarizing current pulses). Each of these measurements depends on membrane potential and on other biophysical properties of the axons. Together they can provide new information about the pathophysiology underlying abnormalities in excitability in neuropathy. 相似文献
63.
de Haan GJ Halley DJ Doelman JC Geesink HH Augustijn PB Jager-Jongkind AD Majoie M Bader AJ Leliefeld-Ten Doeschate LA Deelen WH Bertram E Lehesjoki AE Lindhout D 《Epilepsia》2004,45(9):1061-1063
Summary: Purpose: Univerricht-Lundborg disease (ULD), with its major symptom of action myoclonus, is supposed to be very rare in the Netherlands and western Europe. We hypothesized that the syndrome may be underdiagnosed in patients with myoclonus epilepsy. Methods: Mutation analysis of the cystatin B gene was performed in 21 cases with uncontrolled myoclonus. Results: Seven of the 21 evaluated cases carried mutations in the cystatin B gene. Diagnosis of ULD was made with a mean delay of 20 years from symptom onset. Conclusions: This study from a country without previous reports of ULD suggests that underdiagnosis of the syndrome is likely. These findings also indicate that persons with juvenile-onset myoclonus epilepsy with action myoclonus should be analyzed for ULD. 相似文献
64.
Add-on levetiracetam in children and adolescents with refractory epilepsy: Results of an open-label multi-centre study 总被引:1,自引:0,他引:1
Petra M.C. Callenbach Willem Frans M. Arts Robert ten Houten Paul Augustijn W. Boudewijn Gunning Els A.J. Peeters Alma M. Weber Hans Stroink Yvette Geerts Ada T. Geerts Oebele F. Brouwer 《European journal of paediatric neurology》2008,12(4):321-327
PURPOSE: To study the efficacy and tolerability of add-on levetiracetam in children and adolescents with refractory epilepsy. METHODS: In this prospective multi-centre, open-label, add-on study, 33 children aged 4-16 years (median 8.5 years) with epilepsy refractory to at least two antiepileptic drugs were treated with levetiracetam in addition to their present treatment regimen with a follow-up of 26 weeks. The starting dose of 10 mg/kg/day was increased with 2-week steps of 10 mg/kg/day, if necessary, up to a maximum dose of 60 mg/kg/day. RESULTS: Retention rate was 69.7% after 26 weeks on a median levetiracetam dosage of 22 mg/kg/day. Four children dropped-out because levetiracetam was ineffective, four because seizure frequency increased and/or seizures became more severe, and two because they developed aggressive behaviour. Compared to their baseline seizure frequency, 13 children (39.4%) had a >50% seizure reduction 12 weeks after initiation of levetiracetam, and 17 children (51.5%) at 26 weeks. At 26 weeks, nine children (27.3%) had been seizure-free for at least the last 4 weeks, terminal remission ranged from 0 to 187 days (mean 46 days). Levetiracetam was effective in both partial and primary generalized seizures, but had most effect in partial seizures. Most reported side effects were hyperactivity (48.5%), somnolence (36.4%), irritability (33.3%) and aggressive behaviour (27.3%). Severity of most side effects was mild. Five children had a serious adverse event, which all concerned hospital admissions that were not related to levetiracetam use. CONCLUSION: Levetiracetam proved to be an effective and well-tolerated add-on treatment in this group of children with refractory epilepsy. 相似文献
65.
Pseudomembranous colitis: CT evaluation of 26 cases 总被引:3,自引:0,他引:3
Fishman EK; Kavuru M; Jones B; Kuhlman JE; Merine DS; Lillimoe KD; Siegelman SS 《Radiology》1991,180(1):57
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Background
Infant mortality rate (IMR) is regarded as an important indicator of population health. IMR rates vary substantially with the highest found in sub-Saharan Africa (SSA) compared to the lowest in Europe. Identifying spatial disparities in IMR and quantifying attributable risk factors is essential for policymakers when tailoring time-appropriate interventions at a global, regional, and country level.Methods
Data for 192 countries were extracted from the World Bank Development Indicator database for the period 1990–2011. Spatial clustering was used to identify significant higher-risk IMR countries. A robust ecological generalized linear negative binomial regression model was used to quantify risk factors and associated decomposition values (Shapley).Results
Significant reductions were observed in IMR for all of the World Health Organization regions for the period 1990–2011 except for SSA, which indicated a reversal of this trend in the 1990s due to HIV. Significant high-risk clustering of IMR is also indicated in SSA countries and parts of Asia. Maternal mortality (survival), lack of water and sanitation and female education were confirmed as prominent and high attributable risk factors for IMR. Distinct temporal changes in the attributability of these factors were observed, as well as significant heterogeneity with regards to the most attributable factor by region and country.Conclusions
Our study suggests that maternal mortality is the most prominent attributable risk factor for infant mortality, followed by lack of access to sanitation, lack of access to water, and lower female education. Variation exists across regions and countries with regards to the most attributable factor. Our study also suggests significant underestimation of IMR in regions known for poorer data quality. The results will aid policymakers in re-tailoring time-appropriate interventions to more effectively reduce IMR in line with Millennium Development Goal 4.70.