The parkinsonian LRRK2 R1441G mutation shows macroautophagy-mitophagy dysregulation concomitant with endoplasmic reticulum stress

Cell Biology and Toxicology - Autophagy is a mechanism responsible for the degradation of cellular components to maintain their homeostasis. However, autophagy is commonly altered and compromised...     

Metabolism of glucose 1,6-P2. I. Enzymes involved in the synthesis of glucose 1,6-P2 in pig tissues

Pig tissues show four enzymatic activities of glucose 1,6-P2 synthesis: (A) 2 [glucose 1-P]----glucose 1,6-P2 + glucose; (B) glucose 1-P + ATP----glucose 1,6-P2 + ADP; (C) glucose 1-P + fructose 1,6-P2----glucose 1,6-P2 + fructose 6-P; (D) glucose 1-P + glycerate 1,3-P2----glucose 1,6-P2 + glycerate 3-P. Brain is the tissue with highest capability of glucose 1,6-P2 synthesis. With the exception of skeletal muscle, activity "D" represents the highest activity of glucose 1,6-P2 synthesis. In muscle, activity "B" is the major activity. The existence of a specific glucose 1,6-P2 synthase which catalyzes reaction "D" is confirmed. Two peaks of such an enzyme are isolated by ion-exchange chromatography. There is an enzyme which specifically catalyzes reaction "C", not previously described. There is a glucose 1-P kinase not identical to phosphofructokinase.     

Effect of vanadate on the glucose-1,6-bisphosphate synthase and glucose-1,6-bisphosphatase activities of phosphoglucomutase

Phosphoglucomutase, in addition to catalyzing the interconversion of glucose 1-P and glucose 6-P, catalyzes both the synthesis of glucose 1,6-P2 from glucose monophosphate and either fructose 1,6-P2 or glycerate 1,3-P2, and the hydrolysis of glucose 1,6-P2. Vanadate inhibits the mutase activity, activates the synthase activities, and does not affect the phosphatase activity. These effects suggest that the "exchange" step postulated for the phosphoglucomutase pathway is specifically inhibited by vanadate.     

Purification, characterization and immunological properties of 2,3-bisphosphoglycerate-independent phosphoglycerate mutase from maize (Zea mays) seeds

2,3-Bisphosphoglycerate-independent phosphoglycerate mutase (EC 5.4.2.1) was purified and characterized from maize. SDS electrophoresis showed only one band with a molecular mass of 64 kDa, similar to that determined for the native enzyme by gel-filtration chromatography. The kinetic constants were similar to those reported for wheat germ phosphoglycerate mutase. Rabbit antiserum against maize phosphoglycerate mutase possesses a high degree of specificity. It also reacts with the wheat germ enzyme but fails to react with other cofactor-independent or cofactor-dependent phosphoglycerate mutases. Cell-free synthesis experiments indicate that phosphoglycerate mutase from maize is not post-translationally modified.     

Life histories of Mediterranean pines

The life history of Spanish pines and their relation to fire as the main disturbance factor in their ecosystems was analysed. The primary ecological attributes studied were the canopy seed bank (onset of cone production, percentage and persistence of serotinous cones), seed and cone morphology, sprouting and bark thickness. Four ecological groups were separated using multivariate cluster analysis and their life-history characteristics are discussed. Serotiny and early flowering in Pinus halepensis and P. pinaster reflect their evader strategy in relation to fire as this character is advantageous to survive frequent crown fires and to attain successful post-fire recruitment. Late flowering and absence of serotinous cones in P. nigra, P. sylvestris and P. uncinata indicate that their natural forest did not evolve under frequent crown fires. P. canariensis and P. pinea appeared in two single groups because of their sprouting capability and their seed size respectively. Intraspecific variation in P. pinaster was also analysed using the same criteria and high variability was found in its life history traits. A group of P. pinaster populations showed high levels of serotiny and thin bark as a possible adaptation to frequent stand-replacing crown fires. In contrast, a group of non- or weakly-serotinous populations seems to have evolved under a low-intensity fire regime where the best fitness corresponds to thick-barked individuals capable of surviving ground fires. Intermediate strategies were also evident in this species and were discussed in relation to the effect of different fire regimes caused by the understorey vegetation.     

Effects of thyroid hormone on mRNAs of phosphoglycerate mutase subunits in rat muscle during development

BACKGROUND: We previously showed that triiodothyronine (T3) stimulates muscle phosphoglycerate mutase (PGAM) activity and isozyme transition in rat skeletal and cardiac muscles. METHODS: The effects of T3 on PGAM types B and M subunit expression in rat muscle during development are reported. RESULTS: T3 administration during the first 21 days of rat life more than doubles type M PGAM mRNA levels, but produces minor effects on type B PGAM mRNA levels. The antihormone propylthiouracil (PTU) slightly decreases both type B and M mRNA levels, but this decrease is not statistically significant. CONCLUSION: Thyroid hormone influences PGAM mRNA isozyme levels differently and increases type M mRNA.     

Fetal anasarca (Hydrops foetalis) associated with lymphoid tissue agenesis possibly due to an autosomal recessive gene defect in sheep

Fourteen hydrops fetalis cases appeared in a sheep flock in the Soria province of Central Spain in two lambing seasons in 2000. There were no previous cases of hydrops fetalis in this flock. Normal delivery could not be completed because fetal weights ranged from 12 to 16 kg and fetuses had massive subcutaneous edema. Five affected pregnant females were studied. The complete lack of lymph nodes in the fetuses was the most outstanding finding, this anomaly likely being the origin of generalized fluid accumulation. Karyotypes were normal. A blind protocol of parentage testing was performed by means of DNA microsatellite analysis, and one of the five existing rams was found to be the only compatible sire of the affected fetuses. This male had been selected from the same flock while the other rams had all been acquired from other farms. The first cases appeared when this ram began breeding, and no cases were observed after replacing it. Male and female fetuses were affected in similar proportions. The existence of a recessive allele affecting normal lymph node embryonic development in this flock is proposed as the most appropriate hypothesis. As a consequence, the use of rams from different farms is indicated as an efficient emergency measure in similar situations, while the affected flock should be excluded from selection programs as long as the anomalous gene remains unidentified.