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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.
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 4.68.
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Thrombolytic therapy with tissue plasminogen activator or streptokinase induces transient thrombin activity 总被引:7,自引:0,他引:7
We have determined the plasma level of fibrinopeptide A as a specific index of thrombin activity during the infusion of a thrombolytic agent in patients with acute myocardial infarction. Peripheral venous plasma levels of fibrinopeptide A increased following the initiation of thrombolytic therapy from 2.7 nmol/L to a peak of 13.0 nmol/L at 30 minutes with streptokinase and from 1.1 nmol/L to a peak of 10.7 nmol/L at 90 minutes with tissue plasminogen activator. The amount of fibrinogen converted to fibrin I was determined by integration of the plasma level of fibrinopeptide A over time. The amount of fibrin I formed over the five-hour period from the start of drug infusion was approximately 10 mg/dL in response to either streptokinase or recombinant tissue plasminogen activator. We conclude that activation of coagulation occurs in response to thrombolytic therapy despite heparin administration. This thrombin action, though transient, would be sufficient to cause rethrombosis if localized and incompletely opposed by fibrinolytic activity. 相似文献