Hydroxylated fatty acids are important intermediates in lipid metabolism and signaling. Surprisingly, the metabolism of 4-hydroxy fatty acids remains largely unexplored. We found that both ACAD10 and ACAD11 unite two enzymatic activities to introduce these metabolites into mitochondrial and peroxisomal β-oxidation, respectively.
View Article and Find Full Text PDFLong bone fractures are a concern in long-duration exploration missions (LDEM) where crew autonomy will exceed the current Low Earth Orbit paradigm. Current crew selection assumptions require extensive complete training and competency testing prior to flight for off-nominal situations. Analogue astronauts (n = 6) can be quickly trained to address a single fracture pattern and then competently perform the repair procedure.
View Article and Find Full Text PDFLong bone fractures in hostile environments pose unique challenges due to limited resources, restricted access to healthcare facilities, and absence of surgical expertise. While external fixation has shown promise, the availability of trained surgeons is limited, and the procedure may frighten unexperienced personnel. Therefore, an easy-to-use external fixator (EZExFix) that can be performed by nonsurgeon individuals could provide timely and life-saving treatment in hostile environments; however, its efficacy and accuracy remain to be demonstrated.
View Article and Find Full Text PDFA group of 13 patients with early onset diaphragmatic palsy in association with a progressive neuropathy is presented. All eight of those tested were found to have mutations in the same gene encoding the immunoglobulin mu-binding protein 2 (IGHMBP2) in patients with spinal muscular atrophy (SMA) with respiratory distress type 1. Six out of these eight patients had either homozygous or compound heterozygous mutations, and two had only a single heterozygous mutation.
View Article and Find Full Text PDFMicrovasculitis may play a greater part in the pathogenesis of paraproteinaemic neuropathies than is generally recognised, producing tissue destruction by convergent immune and physical mechanisms. We present a patient with a clinical syndrome of mononeuritis multiplex and a circulating IgM lambda paraprotein, in whom bone marrow aspiration revealed a lymphoplasmacytoid lymphoma. Microvasculitic changes were present in the first nerve biopsy, and the second showed extensive destruction of neural architecture and deposition of IgM-related material.
View Article and Find Full Text PDFFour loose ligatures were tied round the sciatic nerve of rats to produce the model of altered pain sensation first described by Bennett and Xie (1988). Hyperalgesia and hyperaesthesia were detected from 4 days after tying ligatures, becoming maximal after 14 days; normal behaviour returned by 8 weeks post-operation. Using thermal tests involving immersion of the whole foot, it was found that section of the saphenous nerve at the time of, or within a week of, placing ligatures had the effect of abolishing the hyperaesthetic behaviour and instead caused hypoaesthesia from the 4th to 10-12th days.
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