Pseudohypoparathyroidism Type Ib Associated with Novel Duplications in the GNAS Locus

Context Pseudohypoparathyroidism type 1b (PHP-Ib) is characterized by renal resistance to PTH (and, sometimes, a mild resistance to TSH) and absence of any features of Albright's hereditary osteodystrophy. Patients with PHP-Ib suffer of defects in the methylation pattern of the complex GNAS locus. PHP-Ib can be either sporadic or inherited in an autosomal dominant pattern. Whereas familial PHP-Ib is well characterized at the molecular level, the genetic cause of sporadic PHP-Ib cases remains elusive, although some molecular mechanisms have been associated with this subtype. Objective The aim of the study was to investigate the molecular and imprinting defects in the GNAS locus in two unrelated patients with PHP-Ib. Design We have analyzed the GNAS locus by direct sequencing, Methylation-Specific Multiplex Ligation-dependent Probe Amplification, microsatellites, Quantitative Multiplex PCR of Short Fluorescent fragments and array-Comparative Genomic Hybridization studies in order to characterize two unrelated families with clinical features of PHP-Ib. Results We identified two duplications in the GNAS region in two patients with PHP-Ib: one of them, comprising similar to 320 kb, occurred 'de novo' in the patient, whereas the other one, of similar to 179 kb in length, was inherited from the maternal allele. In both cases, no other known genetic cause was observed. Conclusion In this article, we describe the to-our-knowledge biggest duplications reported so far in the GNAS region. Both are associated to PHP-Ib, one of them occurring 'de novo' and the other one being maternally inherited.

A traditional floodplain fishery of the lower Amazon River, Brazil

This paper describes fishing activities of households in four communities located in a floodplain lake system of the lower Amazon river. An average of 42 households were interviewed about their fishing activity on a monthly basis. The fishery is a typical multi-gear, multi-specific artisanal fishery. Approximately ten types of fishing gear are utilized, of which the three main types of gillnets account for 51% of the total catch. The catch per trip averaged 15 kg, for an annual total of 2,295 kg per household. Some 40 species or groups of species are caught, although four species account for 50% of the total. There is a strong seasonal pattern to the fishery, with catch per trip and catch per unit effort (CPUE) highest in the low water season (September-November). While there are marked differences between subsistence and commercially oriented fishing strategies, these differences are more in degree than in type, since fishers use the same types of gear and most fishers regularly sell part of their catch.

Who's working on fishing gear design and construction for small-scale fisheries?

The findings are presented of a search conducted on traditional fishing gear design and construction using the ASFA database (1971-90) and the ICLARM Library and professional staff collections.

Effect of type of otolith and preparation technique on age estimation of larval and juvenile spot (Leiostomus xanthurus)

Otoliths of larval and juvenile fish provide a record of age, size, growth, and development (Campana and Neilson, 1985; Thorrold and Hare, 2002). However, determining the time of first increment formation in otoliths (Campana, 2001) and assessing the accuracy (deviation from real age) and precision (repeatability of increment counts from the same otolith) of increment counts are prerequisites for using otoliths to study the life history of fish (Campana and Moksness, 1991). For most fish species, first increment deposition occurs either at hatching, a day after hatching, or after first feeding and yolksac absorption (Jones, 1986; Thorrold and Hare, 2002). Increment deposition before hatching also occurs (Barkmann and Beck, 1976; Radtke and Dean, 1982). If first increment deposition does not occur at hatching, the standard procedure is to add a predetermined number to increment counts to estimate fish age (Campana and Neilson, 1985).

Quahogs in Eastern North America: Part II, History by Province and State

The northern quahog, Mercenaria mercenaria, ranges along the Atlantic Coast of North America from the Canadian Maritimes to Florida, while the southern quahog, M. campechiensis, ranges mostly from Florida to southern Mexico. The northern quahog was fished by native North Americans during prehistoric periods. They used the meats as food and the shells as scrapers and as utensils. The European colonists copied the Indians treading method, and they also used short rakes for harvesting quahogs. The Indians of southern New England and Long Island, N.Y., made wampum from quahog shells, used it for ornaments and sold it to the colonists, who, in turn, traded it to other Indians for furs. During the late 1600’s, 1700’s, and 1800’s, wampum was made in small factories for eventual trading with Indians farther west for furs. The quahoging industry has provided people in many coastal communities with a means of earning a livelihood and has given consumers a tasty, wholesome food whether eaten raw, steamed, cooked in chowders, or as stuffed quahogs. More than a dozen methods and types of gear have been used in the last two centuries for harvesting quahogs. They include treading and using various types of rakes and dredges, both of which have undergone continuous improvements in design. Modern dredges are equipped with hydraulic jets and one type has an escalator to bring the quahogs continuously to the boats. In the early 1900’s, most provinces and states established regulations to conserve and maximize yields of their quahog stocks. They include a minimum size, now almost universally a 38-mm shell width, and can include gear limitations and daily quotas. The United States produces far more quahogs than either Canada or Mexico. The leading producer in Canada is Prince Edward Island. In the United States, New York, New Jersey, and Rhode Island lead in quahog production in the north, while Virginia and North Carolina lead in the south. Connecticut and Florida were large producers in the 1990’s. The State of Tabasco leads in Mexican production. In the northeastern United States, the bays with large openings, and thus large exchanges of bay waters with ocean waters, have much larger stocks of quahogs and fisheries than bays with small openings and water exchanges. Quahog stocks in certified beds have been enhanced by transplanting stocks to them from stocks in uncertified waters and by planting seed grown in hatcheries, which grew in number from Massachusetts to Florida in the 1980’s and 1990’s.

Quahogs in Eastern North America: Part I, Biology, Ecology, and Historical Uses

The northern quahog, Mercenaria mercenaria, ranges along the Atlantic Coast of North America from the Canadian Maritimes to Florida, while the southern quahog, M. campechiensis, ranges mostly from Florida to southern Mexico. The northern quahog was fished by native North Americans during prehistoric periods. They used the meats as food and the shells as scrapers and as utensils. The European colonists copied the Indians treading method, and they also used short rakes for harvesting quahogs. The Indians of southern New England made wampum from quahog shells, used it for ornaments and sold it to the colonists, who, in turn, traded it to other Indians for furs. During the late 1600’s, 1700’s, and 1800’s, wampum was made in small factories for eventual trading with Indians farther west for furs. The quahoging industry has provided people in many coastal communities with a means of earning a livelihood and has provided consumers with a tasty, wholesome food whether eaten raw, steamed, cooked in chowders, or as stuffed quahogs. More than a dozen methods and types of gear have been used in the last two centuries for harvesting quahogs. They include treading and using various types of rakes and dredges, both of which have undergone continuous improvements in design. Modern dredges are equipped with hydraulic jets and one type has an escalator to bring the quahogs continuously to the boats. In the early 1900’s, most provinces and states established regulations to conserve and maximize yields of their quahog stocks. They include a minimum size, now almost universally a 38-mm shell width, and can include gear limitations and daily quotas. The United States produces far more quahogs than either Canada or Mexico. The leading producer in Canada is Prince Edward Island. In the United States, New York, New Jersey, and Rhode Island lead in quahog production in the north, while Virginia and North Carolina lead in the south. Connecticut and Florida were large producers in the 1990’s. The State of Campeche leads in Mexican production. In the northeastern United States, the bays with large openings, and thus large exchanges of bay waters with ocean waters, have much larger stocks of quahogs and fisheries than bays with small openings and water exchanges. Quahog stocks in certifi ed beds have been enhanced by transplanting stocks to them from stocks in uncertified waters and by planting seed grown in hatcheries, which grew in number from Massachusetts to Florida in the 1980’s and 1990’s.

Temporal Changes in a Tropical Nekton Assemblage and Performance of a Prawn Selective Gear

The temporal variation of components of a moderately diverse (H=1.46) tropical estuarine fish assemblage (long. 146°30'E, lat. 8°45'S) was directed by salinities that had been determined by local oceanographic and probably topographic conditions. For this assemblage, two types of intrayear component profiles are predicted. Pooled data (1988-91) reveal a large component of regular/resident species (43%) in an assemblage which has been under a narrow temperature regime «5T). These results facilitate a discussion on the relevance and usefulness of three hypotheses often cited in studies concerning species diversity and component characteristics of the subtropical/tropical coastal nonreef fish assemblages. Manifestations of the assemblage are reflected in catch composition and weights of 39 trials conducted for a selective prawning gear whose performance in bycatch reduction, mainly for finfishes, is judged by an index, E, we have previously proposed. This gear is capable of harvesting the prawn while conserving the demersal fish. Behavioral responses to netting of the prawns and the finfishes, especially the nearshore surface schoolers such as leiognathids, are discussed from several points of view. An adaptation in terms of group selection for leiognathids of their locking mechanism of median fin spines has been interpreted. For the purpose of bycatch reduction or E enhancement, suggestions for improvements in net design and trawl configuration by considering the behavioral features of fish are made. Our original formula of E is modified for general use. Bycatch problems in the regional prawn fisheries and their possible impacts on fishery planning and development in Papua New Guinea as a developing country are discussed. The gear tested may offer enormous ecological and economic benefits. The gear is multipurpose, extremely simple, and can also be used as a biological sampler.