Backqualität von Winterweizen in Bio- und Extenso Prüfungen

Winter wheat trials have been carried out from 2002 to 2007 under extensive or organic conditions. The results of baking quality analyses have been compared for the same varieties, cultivated under both conditions during the same year. Rheological analyses generally show lower results for the varieties cultivated under organic conditions when baking tests result nearly identical in both networks. Wet gluten is an important baking quality parameter for the users. The percentage of wet gluten is not correlated with the gluten index, the extensographe energy or the points obtained by the baking tests. This parameter alone cannot be used for determination of the baking quality

Cytogenetic studies of Lolium multiflorum Lam., Festuca arundinacea Schreb., their hybrids and amphidiploids

Plant breeders intercross Lolium multiflorum and Festuca arundinacea with the purpose of obtaining hybrids which combine agronomically interesting characters of the parent species. The end result can be an amphidiploid, or the transfer of a limited number of genes from one species to the other. Especially in the first case, meiotic regularity often is a bottle neck. In the present study the influence of various Festuca and Lolium parents on chromosome pairing, further meiotic behaviour and fertility in the hybrids and amphidiploids was studied.In F. arundinacea meiosis is somewhat irregular due to the presence of univalents and multivalents and lack of stability. For the same plant significant differences in number of chromosome. arms bound by chiasmata were found over two years, and a number of plants even showed significant differences within clones within years. Significant differences in meiotic chromosome association were found between the Festuca varieties. Between plants of the same variety, differences were also found, but were smaller than between varieties. Chromosome association seems to have little effect on pollen stainability and fertility.Diploid Lolium had very regular meiosis and most of the chromosomes formed ring-bivalents at first metaphase. Tetraploid Lolium showed some uni- and trivalents, and quadrivalents were found with very low frequency (average 1.24 per cell), for an autotetraploid.Hybrids were obtained easily between diploid L. multiflorum varieties Lior and Manawa (as female parent) and F. arundinacea . The percentage of seed set was high: 44% for the cross Lior x Festuca and 50% for Manawa x Festuca. Some differences in seed set between the Festuca parents were found with Ludion as the variety with the highest seed set. Diploid Lolium varieties did not influence seed set but influenced germination. The crosses with the variety Manawa had a significantly higher percentage of germination (76%) than the crosses with Lior (50%). The reciprocal cross gave a very low percentage seed set (0.75%), and only one hybrid seed germinated. The seed set of the crosses with the tetraploid Lolium as female parent was much lower (19%) than that of the diploid Lolium and the germination of the hybrid seeds was very low.The hybrids (2n = 28) showed a high level of meiotic chromosome association and averages of 10.31 bivalents for the hybrids Lior x Festuca and 10.68 bivalents for Manawa x Festuca were found. Univalents, tri- and quadrivalents were observed frequently. The differences between the hybrids in numbers of univalents and numbers of chromosome arms bound by chiasmata were highly significant, but no specific influence of the Festuca nor the Lolium parent could be detected. Pollen stainability was very low and the hybrids were completely male sterile. Pollen development was almost normal until first pollen mitosis, after which the pollen grains degenerated. This might be due to a genetic unbalance and it is possible that pollen grains completing first pollen mitosis had all seven Lolium chromosomes present.In the amphidiploids most of the chromosomes paired as bivalents (average 22.29 for the amphidiploids Lior x Festuca and 24.31 for Manawa x Festuca). In comparison to the chromosome pairing in the hybrids a higher number of multivalents could be expected. This was not the case and pairing was mostly limited to bivalents. All amphidiploids showed univalents and multivalents. As for the hybrids, significant differences in numbers of univalents and of arms bound were found between the amphi-diploids. As observed for the hybrids, the chromosome pairing in the amphidiploids depended on the combination of the parents rather than on the specific genotype of the varieties used. Chromosome pairing in the amphidiploids was not influenced by temperature in the range between 15 and 25 °C. C 1 plants with 56 chromosomes did not show a more regular meiosis than C 0 plants.Comparison between the tetraploid and pentaploid (from tetraploid Lolium x Festuca) hybrids with their corresponding amphidiploids showed that although most amphidiploids had a higher number of arms bound per chromosome than their hybrid, only for four octoploid amphidiploids the differences were significant. Chromosome pairing thus increased only slightly or not at all (decaploids) after chromosome doubling.The number of micronuclei per tetrad (M/T) proved to be a highly variable character and was not always correlated with the number of univalents.However, this character can still be useful for selection. Plants with a very low number of M/T perhaps have a low number of univalents but most probably a relatively good pollen stainability, gemination of the pollen grains and fertility.Comparison between the Festuca parents, the hybrids and the amphidiploids in respect to the number of arms bound per chromosome (table 23) showed no significant differences between the parents and the hybrids and between the parents and the amphidiploids. For the same number of arms bound per chromosome, Festuca had a more regular meiosis with only homologous pairing and the amphidiploids an irregular meiosis with mainly homologous but probably also homoeologous pairing, or no pairing at all. It is likely that chromosome pairing in F. arundinacea , which is restricted to bivalents, is under genetic control. It seems that the Lolium genome partially suppresses the action of the pairing regulating genes of F. arundinacea . This could be the cause of the meiotic instability of the amphidiploids.Calculation of the relative affinity showed that in the hybrids two genomes are more closely related to each other than they are to a similarly related second pair of genomes, The Lolium genome is closely related to the genome of F. pratensis , which probably is the donor of one of the genomes of F. arundinacea . The two other genomes of F. arundinacea are closely related so that in haploid state, their chromosomes can pair.In this study, even with very distinct Festuca parents and significant differences in chromosome pairing, no amphidiploid with a stable meiosis was found. The fact that between the amphidiploids significant differences in numbers of arms bound were observed may indicate that certain genetic combinations give a regular meiosis but the chance of finding such a genotype is very small. If the amphidiploids cannot be used directly for breeding purposes, introgression of some characters from one into the other parental species seems to be the most promising way of using the amphidiploids between L. multiflorum and F. a rundinacea . Since homoeologous pairing occurs, introgression is possible

An early-onset recessive cerebellar disorder with distal amyotrophy and, in two patients, gross myoclonia: A probable ataxia telangiectasia variant

We report a family of 4 siblings from a non-consanguineous marriage, presenting with an early onset recessive cerebellar ataxia and progressive distal limb wasting. Ocular or other telangiectasias were absent. There were neither frequent infections nor immunodeficiencies. The two youngest patients exhibited an incapacitating myoclonus which abated markedly after 20 years. Late onset diabetes was demonstrated in 3 patients. Hypogonadism was not a feature and there was a prolonged survival in the 4 patients. The oldest sibling died of a pancreatic adenocarcinoma. α-Fetroprotein was elevated with normal carcinoembryonic antigen values in three patients. Cytogenetic analysis and radioresistant DNA synthesis was compatible with the diagnosis of ataxia-telangiectasia. This family probably represents a rare variant of ataxia-telangiectasia

Stalboekje varkens : natuurlijk gezond met kruiden en andere natuurproducten

Het streven om het gebruik van antibiotica terug te dringen vraagt om een ander management. Goede voeding, huisvesting en hygiëne zijn hierbij belangrijk. In dit boekje worden handvaten gegeven om met natuurlijke middelen de gezondheid van de dieren te bevorderen en zo ziektes te voorkomen. Tevens kunnen middelen worden ingezet om de ernst van de ziekte te reduceren. Doel is tevens om de dierenartsen te informeren over de mogelijkheden van natuurproducten en de wetenschappelijke onderbouwing hiervan inzichtelijk te make

A Critical Tryptophan and Ca2+ in Activation and Catalysis of TPPI, the Enzyme Deficient in Classic Late-Infantile Neuronal Ceroid Lipofuscinosis

Tripeptidyl aminopeptidase I (TPPI) is a crucial lysosomal enzyme that is deficient in the fatal neurodegenerative disorder called classic late-infantile neuronal ceroid lipofuscinosis (LINCL). It is involved in the catabolism of proteins in the lysosomes. Recent X-ray crystallographic studies have provided insights into the structural/functional aspects of TPPI catalysis, and indicated presence of an octahedrally coordinated Ca(2+).Purified precursor and mature TPPI were used to study inhibition by NBS and EDTA using biochemical and immunological approaches. Site-directed mutagenesis with confocal imaging technique identified a critical W residue in TPPI activity, and the processing of precursor into mature enzyme.NBS is a potent inhibitor of the purified TPPI. In mammalian TPPI, W542 is critical for tripeptidyl peptidase activity as well as autocatalysis. Transfection studies have indicated that mutants of the TPPI that harbor residues other than W at position 542 have delayed processing, and are retained in the ER rather than transported to lysosomes. EDTA inhibits the autocatalytic processing of the precursor TPPI.We propose that W542 and Ca(2+) are critical for maintaining the proper tertiary structure of the precursor proprotein as well as the mature TPPI. Additionally, Ca(2+) is necessary for the autocatalytic processing of the precursor protein into the mature TPPI. We have identified NBS as a potent TPPI inhibitor, which led in delineating a critical role for W542 residue. Studies with such compounds will prove valuable in identifying the critical residues in the TPPI catalysis and its structure-function analysis

Diagnosing mucopolysaccharidosis IVA

Mucopolysaccharidosis IVA (MPS IVA; Morquio A syndrome) is an autosomal recessive lysosomal storage disorder resulting from a deficiency of N-acetylgalactosamine-6-sulfate sulfatase (GALNS) activity. Diagnosis can be challenging and requires agreement of clinical, radiographic, and laboratory findings. A group of biochemical genetics laboratory directors and clinicians involved in the diagnosis of MPS IVA, convened by BioMarin Pharmaceutical Inc., met to develop recommendations for diagnosis. The following conclusions were reached. Due to the wide variation and subtleties of radiographic findings, imaging of multiple body regions is recommended. Urinary glycosaminoglycan analysis is particularly problematic for MPS IVA and it is strongly recommended to proceed to enzyme activity testing even if urine appears normal when there is clinical suspicion of MPS IVA. Enzyme activity testing of GALNS is essential in diagnosing MPS IVA. Additional analyses to confirm sample integrity and rule out MPS IVB, multiple sulfatase deficiency, and mucolipidoses types II/III are critical as part of enzyme activity testing. Leukocytes or cultured dermal fibroblasts are strongly recommended for enzyme activity testing to confirm screening results. Molecular testing may also be used to confirm the diagnosis in many patients. However, two known or probable causative mutations may not be identified in all cases of MPS IVA. A diagnostic testing algorithm is presented which attempts to streamline this complex testing process

Packages of Care for Dementia in Low- and Middle-Income Countries

In the fifth in a series of six articles on packages of care for mental disorders in low- and middle-income countries, Martin Prince and colleagues discuss the treatment of dementia

Pompe disease diagnosis and management guideline

ACMG standards and guidelines are designed primarily as an educational resource for physicians and other health care providers to help them provide quality medical genetic services. Adherence to these standards and guidelines does not necessarily ensure a successful medical outcome. These standards and guidelines should not be considered inclusive of all proper procedures and tests or exclusive of other procedures and tests that are reasonably directed to obtaining the same results. in determining the propriety of any specific procedure or test, the geneticist should apply his or her own professional judgment to the specific clinical circumstances presented by the individual patient or specimen. It may be prudent, however, to document in the patient's record the rationale for any significant deviation from these standards and guidelines.Duke Univ, Med Ctr, Durham, NC 27706 USAOregon Hlth Sci Univ, Portland, OR 97201 USANYU, Sch Med, New York, NY USAUniv Florida, Coll Med, Powell Gene Therapy Ctr, Gainesville, FL 32611 USAIndiana Univ, Bloomington, in 47405 USAUniv Miami, Miller Sch Med, Coral Gables, FL 33124 USAHarvard Univ, Childrens Hosp, Sch Med, Cambridge, MA 02138 USAUniversidade Federal de São Paulo, São Paulo, BrazilColumbia Univ, New York, NY 10027 USANYU, Bellevue Hosp, Sch Med, New York, NY USAColumbia Univ, Med Ctr, New York, NY 10027 USAUniversidade Federal de São Paulo, São Paulo, BrazilWeb of Scienc