bluuebunny/crossref_metadata_2025 huggingface dataset:
Lowβ‐glucuronidase activity in a healthy member of a family with mucopolysaccharidosis VII
Authors: Chabas A. , Giros M. L. , Guardiola A.
Published: June 2005

A phenotypically normal mother of a mucopolysaccharidosis VII child, is reported with an unusually lowβ‐glucuronidase activity. Low enzyme activity was systemic (6–10% of controls) and residualβ‐glucuronidase in leukocytes had an apparently normalKm value. [35S]sulphate incorporation and chase assays in fibroblasts gave values similar to control cells. A normal excretion pattern of glycosaminoglycan was found in this woman's urine. Low enzymatic activity can be related to a non‐pathological ‘pseudodeficiency’ allele forβ‐glucuronidase; this woman appears to be an apparent compound heterozygote for this allele and mucopolysaccharidosis VII. Her next pregnancy was monitored by chorionic villus sampling and a heterozygous fetus was suspected. These studies stress the need for complete enzyme investigations of obligate carriers for mucopolysaccharidoses in order to prevent difficulties at prenatal analysis.
Prenatal diagnosis of molybdenum cofactor deficiency by assay of sulphite oxidase activity in chorionic villus samples
Authors: Johnson J. L. , Rajagopalan K. V. , Lanman J. T. , Schutgens R. B. H. , van Gennip A. H. , Sorensen P. , Applegarth D. A.
Published: June 2005

Molybdenum cofactor deficiency is characterized by the absence of sulphite oxidase, xanthine dehydrogenase and aldehyde oxidase, the three known enzymes in man that require the cofactor for their activity. Prenatal diagnosis of the deficiency may be performed by assay of sulphite oxidase activity in cultured amniocytes. However, the activity in amniocytes is low and large numbers of cells are required for reliable assessment. We show that sulphite oxidase is present at high levels in chorionic villi obtained at 10–14 weeks gestation and can be assayed directly in the biopsy sample without cell culture. This assay has been applied to two pregnancies at risk for molybdenum cofactor deficiency with successful diagnoses of an unaffected and an affected fetus.
Age‐related differences in plasmalogen content of erythrocytes from patients with the cerebro‐hepato‐renal (Zellweger) syndrome: Implications for postnatal detection of the disease
Authors: Wanders R. J. A. , Purvis Y. R. , Heymans H. S. A. , Bakkeren J. A. J. M. , Parmentier G. G. , van Eldere J. , Eyssen H. , van den Bosch H. , Tager J. M. , Schutgens R. B. H.
Published: June 2005

Phosphatidylethanolamine plasmalogen levels were determined in erythrocytes from controls and 13 patients with the cerebro‐hepato‐renal (Zellweger) syndrome. It was found that in Zellweger patients 20 weeks of age or younger, erythrocyte phosphatidylethanolamine plasmalogen levels were lowered whereas in older patients (except in one) normal levels were found. The results obtained suggest a close relationship between the age of the patients at sampling and the phosphatidylethanolamine plasmalogen levels in their erythrocytes. A possible explanation for these findings and the implications for the postnatal detection of Zellweger syndrome are discussed.
The biochemistry of lipoproteins
Authors: Salter A. M. , Brindley D. N.
Published: June 2005

Lipids are transported in the blood in four major classes of lipoproteins. The triacylglycerol‐rich lipoproteins are chylomicrons and very‐low‐density lipoproteins (VLDL) which are produced by the small intestine and liver, respectively. These lipoproteins mainly carry fatty acids to adipose tissue and muscle where the triacylglycerol is hydrolysed by lipoprotein lipase. The resulting particles that remain in the blood are chylomicron remnants and low‐density lipoprotein (LDL), respectively. The remnant is taken up by the liver via endocytosis which is mediated by a specific receptor for apolipoprotein E (apoE). LDL, which are rich in cholesterol, can also be taken up by the liver or extrahepatic tissues by a receptor‐mediated endocytosis that specifically recognises apoB or apoE. ‘Nascent’ high‐density lipoprotein (HDL) particles are secreted by the liver and intestine and then undergo modification to become HDL3 and then HDL2 as they acquire cholesterol ester. They facilitate the reverse transport of cholesterol back to the liver.

Little is known of the hormonal regulation of lipoprotein uptake by the liver. Recently, we have shown that insulin and tri‐iodothyronine (T3) increase the specific binding of LDL to cultured hepatocytes whereas dexamethasone (a synthetic glucocorticoid) has the opposite effect. The changes in binding produced by insulin and dexamethasone are paralleled by alterations in the rate of degradation of apoB. These findings may in part explain the hypercholesterolaemia and increased risk of premature atherosclerosis that can be associated with poorly controlled diabetes or hypothyroidism.
Lipase deficiencies
Authors: Greten H. , Beil F. U.
Published: June 2005

Two enzymes, lipoprotein lipase and hepatic triglyceride lipase, are involved in the hydrolysis of triglycerides from chylomicrons and very low density lipoprotein (VLDL). Lipoprotein lipase has an absolute requirement for apolipoprotein CII for activity. Three inborn errors of metabolism which give rise to hypertriglyceridaemia have been described. The biochemical and clinical aspects of these disorders, lipoprotein lipase deficiency (familial type I hyperlipoproteinaemia), hepatic triglyceride lipase deficiency and apo‐CII deficiency are discussed.
Familial LCAT deficiency and fish‐eye disease
Authors: McIntyre N.
Published: June 2005

Familial LCAT deficiency is due to deficiency of plasma lecithin‐cholesterol acyltransferase. The plasma is rich in free cholesterol and lecithin while cholesterol ester and lysolecithin levels are reduced. Analysis of the abnormal lipoproteins has helped our understanding of plasma lipid and lipoprotein metabolism in normals and in patients with liver disease.

Proteinuria and anaemia are common and there is marked corneal lipid deposition. Eventually renal function deteriorates and dialysis and/or renal transplantation may be necessary.

The human LCAT gene has been sequenced and been shown to be present on chromosomal segment 16q22 — the region predicted on the basis of recombination studies as the site of the LCAT deficiency gene. The gene defect has been identified in some cases, but the mechanism remains unclear as the mutations were not in the region presumed to be the enzyme's active site.

Only three cases of fish‐eye disease have been described; all were elderly and had obvious corneal opacities. They had fasting hypertriglyceridaemia and increased VLDL. IDL and LDL were increased and were triglyceride rich. HDL, reduced by 90%, was mainly HDL3 — with a high free and low ester cholesterol.

LCAT activity in fish‐eye plasma was normal but when measured in an exogenous substrate it was only 10–15% of normal. Fish‐eye HDL is a substrate for purified LCAT, but fish‐eye LCAT does not esterify free cholesterol of HDL (normal or fish‐eye), although it esterifies free cholesterol of VLDL and LDL. It has been suggested that one type of LCAT activity acts on HDL (α‐LCAT) and another on VLDL and LDL (β‐LCAT) — and that fish‐eye disease is due toα‐LCAT deficiency, and classical familial LCAT deficiency due to lack of both components.
Biochemical, clinical, genetic and metabolic studies of hyperapo‐β‐lipoproteinaemia
Authors: Kwiterovich P. O.
Published: June 2005

Hyperapo‐β‐lipoproteinaemia is a common lipoprotein disorder characterized by an elevated plasma level of the major apolipoprotein, B (apoB) of low‐densityβ lipoproteins (LDL), combined with a low ratio of LDL cholesterol to LDL apoB. Hyperapo‐β‐lipoproteinaemia is due to the overproduction of LDL apoB that results from an enhanced synthesis of very low‐density (pre‐β) lipoprotein (VLDL) in liver. The plasma levels of high‐density (α) lipoprotein (HDL) and its major apolipoprotein, A‐I, are often low in hyperapo‐β‐lipoproteinaemia. Hyperapo‐β‐lipoproteinaemia is often familial and aggregates in children and adults from families with premature coronary artery disease. The precise defect(s) that cause hyperapo‐β‐lipoproteinaemia are not known. In a family with premature coronary artery disease and hyperapo‐β‐lipoproteinaemia, a mutation in codon 4046 in exon 29 of the apolipoprotein B gene, a CGG to TGG transition produced a change from arginine, a positively charged amino acid, to tryptophan, a hydrophobic amino acid, at position 4,019 of the mature apolipoprotein B protein. Decreased incorporation of free fatty acids into triglycerides of adipocytes has been describedin vitro, andin vivo studies suggested a defect in clearance of postprandial lipoproteins associated with decreased uptake of plasma free fatty acids.
Recent advances in cystic fibrosis
Authors: McPherson M. A.
Published: June 2005

Cystic fibrosis, one of the most common lethal inherited disorders in N. European and N. American populations, is characterized by the production of abnormally viscous mucous secretions in the lungs and digestive tract. The pathophysiological basis of the disease is unknown. However, during the last few years, rapid advances in molecular genetics and biochemical and physiological studies on cystic fibrosis epithelial cells have led to optimism that the cystic fibrosis defect will soon be identified. Current evidence suggests that the basic disturbance lies in altered regulation of protein secretion and electrolyte transport leading to an imbalance in composition of epithelial secretions in cystic fibrosis patients. Increasing knowledge of the mechanisms regulating production and secretion of mucins and movement of electrolytes across the cell membrane should lead to development of pharmacological manipulation(s) to correct the cellular abnormality. Ultimately, it is hoped that this will lead to the development of a rational treatment for cystic fibrosis patients.
Determination of serum apolipoprotein B by competitive enzyme‐linked immunoassay
Authors: Reisinger H. , Strobl W. , Hubrich B. , Widhalm K.
Published: June 2005

An enzyme‐linked immunoassay was used to determine apolipoprotein B (apoB) in serum samples from 104 healthy adults aged 18–26 years. The method was compared to radial immunodiffusion (RID). Mean apoB values were 103±30 mg/dl and 105±31 mg/dl respectively. The correlation coefficient for apoB between the two methods wasr=0.70,p<0.001; the correlation coefficient between LDL‐cholesterol and apoB measured by the ELISA wasr=0.73,p<0.001.

This ELISA technique seems to be an appropriate method for measuring apoB in plasma, in the clinical laboratory and for detecting individuals with hyperapobetalipoproteinaemia.
Evaluation of cofactor responsiveness
Authors: Leonard J. V. , Daish P.
Published: June 2005

The response to cofactor therapy in inborn errors of metabolism may be dramatic with complete resolution of the clinical illness but more commonly the response is absent or partial. When assessing cofactor responsiveness clinical and biochemical findings as well as the natural history of the disorder must be taken into account.
Clinical role of pteridine therapy in tetrahydrobiopterin deficiency
Authors: Smith I. , Hyland K. , Kendall B. , Leeming R.
Published: June 2005

In most patients with deficiency of tetrahydrobiopterin (BH4) continuous administration of BH4 or of a synthetic analogue such as 6‐methyltetrahydropterin (6‐MPH4) lowers plasma phenylalanine concentrations to the therapeutic range. The effective dose of BH4 varies from 1 to 2 mg kg−1 daily in patients with defective biopterin synthesis, to 5 mg kg−1 or more in patients with dihydropteridine reductase (DHPR) deficiency. The cost of 2 mg kg−1 day−1 of BH4 is comparable to the cost of a low phenylalanine diet.

Higher doses of pterins given orally (20 mg kg−1) raise the levels of tetrahydropterin in cerebrospinal fluid (CSF) to normal in patients with defective biopterin synthesis in whom initial concentration of biopterin species are low. In some, but not all, such patients pterin therapy also raises CSF amine metabolite concentrations and ameliorates symptoms. High dose therapy does not appear to be effective in raising CSF pterin levels in patients with DHPR deficiency who already accumulate dihydrobiopterin (BH2) in CSF.

Central folate deficiency is an additional cause of neurological deterioration in patients with DHPR deficiency who require supplementation with folate as folinic acid. It is suggested that the accumulation of BH2 in such patients competitively interferes with folate metabolism.
Thiamine‐responsive inborn errors of metabolism
Authors: Duran M. , Wadman S. K.
Published: June 2005

Three different inherited disorders are known in which thiamine may exert a beneficial effect: maple syrup urine disease (MSUD), lactic acidaemia and the syndrome of megaloblastic anaemia with sensorineural deafness and diabetes mellitus. The amounts of thiamine which were used for long‐term treatment varied from 20 to 2400 mg day−1. Additional treatment, such as the reduction of dietary branched chain amino acids in MSUD, could not be omitted in some cases. It has been shown that the vitamin improves the stability of the branched chain ketoacid decarboxylase, although some weeks may be needed to observe thein vivo effect of treatment. A prolonged trial with high doses of thiamine should always be given.
RFLPs of the phenylalanine hydroxylase gene in the Italian population
Authors: Dianzani I. , Farinasso L. , Fortina P. , Camaschella C. , Ponzone R. , Dahl H‐H. M. , Cotton R. G. H. , Ponzone A.
Published: June 2005

Different mutations of the phenylalanine hydroxylase (PAH) gene leading to phenylketonuria (PKU) have been described associated with specific haplotypes in several European countries. In order to investigate the distribution of DNA haplotypes in Italy, restriction fragment length polymorphism (RFLP) analysis of the PAH gene was performed in nine Italian PKU patients from eight unrelated families, and in the available relatives. The analysis of eight polymorphic sites revealed haplotypes 1 and 6 in association with PKU. This pattern appears to differ from those reported for other European populations.

The majority of the 14 PKU subjects studied showed compound heterozygosity for different haplotypes, as observed for other European series.

RFLP analysis at the PAH locus allowed us to offer the possibility of prenatal diagnosis to six of the studied families. One prenatal diagnosis was performed and a normal fetus was diagnosed.
DNA analysis for ornithine transcarbamylase deficiency
Authors: Rozen R. , Fox J. E. , Hack A. M. , Fenton W. A. , Horwich A. L. , Rosenberg L. E.
Published: June 2005

We have utilized the Southern blotting technique to analyse genomic DNA from males with ornithine transcarbamylase (OTC) deficiency and their families. Using a nearly full‐length human cDNA probe, we have identified 3 patients with deletions at this locus and have characterized 4 different restriction fragment length polymorphisms that can be used as linkage markers for the OTC mutation. These polymorphisms occur at sufficiently high frequencies so as to enable us to distinguish the two X‐chromosomes in approximately 80% of OTC carriers. As a direct consequence of these findings, prenatal diagnosis and carrier assessment can be offered to a large fraction of families at risk for OTC deficiency.
Quinolinate synthetase, an iron–sulfur enzyme in NAD biosynthesis
Authors: Ollagnier-de Choudens Sandrine , Loiseau Laurent , Sanakis Yiannis , Barras Frédéric , Fontecave Marc
Published: June 2005

Nicotinamide adenine dinucleotide (NAD) plays a crucial role as a cofactor in numerous essential redox biological reactions. NAD derives from quinolinic acid which is synthesized in Escherichia coli from l‐aspartate and dihydroxyacetone phosphate (DHAP) as the result of the concerted action of two enzymes, l‐aspartate oxidase (NadB) and quinolinate synthetase (NadA). We report here the characterization of NadA protein from E. coli. When anaerobically purified, the isolated soluble protein contains 3–3.5 iron and 3–3.5 sulfide/polypeptide chain. Mössbauer spectra of the 57Fe‐protein revealed that the majority of the iron is in the form of a (4Fe–4S)2+ cluster. An enzymatic assay for quinolinate synthetase activity was set up and allowed to demonstrate that the cluster is absolutely required for NadA activity. Exposure to air leads to degradation of the cluster and inactivate enzyme.
Translational repression by the cis‐acting element of structure‐anchored repression (CAESAR) of human ctgf/ccn2 mRNA
Authors: Kubota Satoshi , Mukudai Yoshiki , Moritani Norifumi H. , Nakao Kyouji , Kawata Kazumi , MasaharuTakigawa
Published: June 2005

The cis‐acting element of structure‐anchored repression (CAESAR) is a post‐transcriptional regulatory element of gene expression, which is located in the 3′‐untranslated region (UTR) of the human ccn2 gene (ctgf/ccn2). In this report, the repression mechanism of CAESAR, as well as the structural requirement, was investigated. Removal of minor stem‐loops from CAESAR resulted in proportional attenuation of the repressive function, whereas removal of the single bulge or modification of primary nucleotide sequence did not affect its functionality. In light of functional mechanism, CAESAR exerted no significant effects on stability or nuclear export of the cis‐linked mRNA. However, this element significantly interfered with the association of such mRNA on ribosome and slowed down the translation process thereafter in vitro. A translation repression mechanism by RNA secondary structure to determine the basal ctgf/ccn2 expression level was uncovered herein.
The yeast osmosensitive mutant fps1Δ transformed by the cauliflower BobTIP1;1 aquaporin withstand a hypo‐osmotic shock
Authors: Prudent Sébastien , Marty Francis , Charbonnier Maryse
Published: June 2005

Osmoregulation plays an important role in cellular responses to osmotic stress in plants and in yeast. Aquaporins contribute to osmotic adjustment by facilitating transport of water or solutes across membranes. The tonoplastic water channel BobTIP1;1 (original name BobTIP26‐1) genes are upregulated during dessication stress in cauliflower meristematic tissue. To investigate the physiological importance of BobTIP1;1, we expressed it in a Saccharomyces cerevisiae osmosensitive mutant fps1Δ. We showed that the defect in the yeast glycerol plasma membrane transporter is complemented by a plant cDNA encoding the aquaporin BobTIP1;1 which is localized in the vacuolar membrane of the complemented yeast cells. To our knowledge, this is the first example of a plant aquaporin for which localization in the vacuolar membrane of yeast cells is related to an osmoresistant phenotype under hypo‐osmotic shock.
Parkin interacts with the proteasome subunit α4
Authors: Dächsel J.C. , Lücking C.B. , Deeg S. , Schultz E. , Lalowski M. , Casademunt E. , Corti O. , Hampe C. , Patenge N. , Vaupel K. , Yamamoto A. , Dichgans M. , Brice A. , Wanker E.E. , Kahle P.J. , Gasser T.
Published: June 2005

Mutations in the parkin gene encoding an E3 ligase are responsible for autosomal recessive Parkinson's disease. Putative parkin substrates and interacting partners have been identified, but the molecular mechanism underlying parkin‐related neurodegeneration is still unclear. We have identified the 20S proteasomal subunit α4 (synonyms: PSMA7, XAPC7, subunit alpha type 7) as a new interacting partner of parkin. The C‐terminal IBR‐RING domain of parkin and the C‐terminal part of α4 were essential for the interaction. Biochemical studies revealed that α4 was not a substrate for parkin‐dependent ubiquitylation. Putative functions of the interaction might therefore be substrate presentation to the proteasome or regulation of proteasomal activity. Full‐length parkin and parkin lacking the N‐terminal ubiquitin‐like domain slightly increased the proteasomal activity in HEK 293T cells, in line with the latter hypothesis.
Survival probability of Baltic larval cod in relation to spatial overlap patterns with their prey obtained from drift model studies
Authors: Hinrichsen H-H. , Schmidt J.O. , Petereit C. , Möllmann C.
Published: June 2005

Temporal mismatch between the occurrence of larvae and their prey potentially affects the spatial overlap and thus the contact rates between predator and prey. This might have important consequences for growth and survival. We performed a case study investigating the influence of circulation patterns on the overlap of Baltic cod larvae with their prey. A three-dimensional hydrodynamic model was used to analyse spatio-temporally resolved drift patterns of larval Baltic cod. A coefficient of overlap between modelled larval and idealized prey distributions indicated the probability of predator–prey overlap, dependent on the hatching time of cod larvae. By performing model runs for the years 1979–1998 investigated the intra- and interannual variability of potential spatial overlap between predator and prey. Assuming uniform prey distributions, we generally found the overlap to have decreased since the mid-1980s, but with the highest variability during the 1990s. Seasonally, predator–prey overlap on the Baltic cod spawning grounds was highest in summer and lowest at the end of the cod spawning season. Horizontally variable prey distributions generally resulted in decreased overlap coefficients. Finally, we related variations in overlap patterns to the variability of Baltic cod recruitment success.
FOREWORD
Authors: Bone Quentin
Published: June 2005

This special issue of JMBA papers on the gelatinous plankton is dedicated to Sir Frederick Russell, Director of the Plymouth Laboratory of the Marine Biological Association from 1945 to 1965 and pioneer in the study of gelatinous plankton.
Climate effects on formation of jellyfish and ctenophore blooms: a review
Authors: Purcell Jennifer E.
Published: June 2005

Much speculation and some evidence suggest that jellyfish and ctenophore populations have increased in recent decades. Unfortunately, few past records exist with which to compare current populations, and our knowledge of how environmental factors affect jellyfish population size is meagre. Human enterprise has wrought many changes in the ocean that are hypothesized to favour jellyfish, including eutrophication, reduction of fish stocks, and global warming. In addition to anthropogenic changes, natural climate cycles may affect jellyfish populations. Records of jellyfish and ctenophore abundance that appear to be related to indices of climate variations (temperature, salinity, North Atlantic Oscillation, North Pacific Decadal Oscillation, El Niño Southern Oscillation) are reviewed. In eleven species studied from subtropical, temperate and subarctic environments, warm temperatures were related to large population sizes; three scyphozoan species in the North Sea, and one mesopelagic hydromedusan were exceptions to that trend. One tropical scyphomedusan species was decimated by unusually warm, salty El Niño conditions in Palau. Because climate changes have complex ecosystem-level effects, the proximate causes of jellyfish increases are difficult to deduce. Therefore, the effects of temperature, salinity and prey on asexual production of new medusae from the benthic polyps of scyphomedusae and hydromedusae also are reviewed. Experiments on temperate species show greater and more rapid production of medusae at warmer temperatures. Salinity also had significant effects, and was especially important for estuarine species. Temperature and salinity affect asexual reproduction rates directly through metabolism, and indirectly through prey capture. Ocean warming may shift the distributions, expand the seasonal occurrence, and increase the abundances of temperate-boreal species. Populations living near their thermal maximum may suffer negative consequences of warming.
Reproduction and longevity of Aurelia labiata in Roscoe Bay, a small bay on the Pacific coast of Canada
Authors: Albert D.J.
Published: June 2005

Reproduction by the moon jelly, Aurelia labiata, was observed in a small bay on the west coast of Canada. The bay is located in an area isolated from human contact except in the summer, is sheltered from wind and wave action by mountain ridges, and has limited tidal flushing due to a gravel bar at the entrance that dries at lower low water. Planulae appeared in brood sacs beginning in October and November. The planulae were shed by the end of March and ephyrae emerged in June. Juvenile medusae were estimated to constitute about 30—40% of medusae in the bay in each of the two years of this study. Medusae remained in the bay throughout the year. There was no major visible mortality in the adult population during the two year observation period. Medusae appear to be lost from the bay as a result of tidal flushing. It is argued that in Roscoe Bay Aurelia labiata medusae live for more than one year and that up to 40% of the adult medusae may be two years of age or older.
Re-description of Rosacea cymbiformis, a prayine siphonophore
(from the Mediterranean Sea), with comments on nectophore
designation and bract orientation
Authors: Mapstone Gillian M.
Published: June 2005

This updated re-description of the prayine siphonophore Rosacea cymbiformis includes figures of all zooids (except larval nectophores) and is based on material held in the collections of the Natural History Museum (NHM), London. Rosacea cymbiformis was originally described in 1830 under the name Physalia cymbiformis, and subsequently reported many times during the 19th Century. However, during the 20th Century it was confused with the closely related species R. plicata, and the two species are still not clearly differentiated. Previous descriptions are reviewed herein, including conflicting interpretations of nectophore designation in R. plicata, and bract orientation in R. cymbiformis and R. plicata. To identify these siphonophores to species level and separate them from other closely related prayines, it is essential to distinguish the first definitive nectophore from the second, and the right paired bracteal canals from the left canals. This becomes critical when only detached siphonophore zooids are available, as for example, in plankton samples collected with nets. A summary of the differences between R. cymbiformi and the five other currently recognized Rosacea species, R. plicata, R. repanda, R. limbata, R. flaccida and R. arabiana, is presented. The full synonymy of R. cymbiformis is too long for inclusion here and is deferred to a later paper.
Morphologic and molecular redescription of
Catostylus mosaicus conservativus (Scyphozoa: Rhizostomeae: Catostylidae) from south-east Australia
Authors: Dawson Michael N
Published: June 2005

Two reciprocally monophyletic mitochondrial clades of the commercially valuable jellyfish Catostylus mosaicus are endemic to south-eastern Australia. Here, medusae in the two clades are shown to differ also in colour and in the dimensions of their papillae, oral disk, and bell depth. They are referred to two varieties recognized in 1884 by von Lendenfeld. The clade occupying localities adjacent to Bass Strait is redescribed as subspecies C. mosaicus conservativus; the clade from New South Wales and southern Queensland spans the type locality (Port Jackson) of C. mosaicus and is designated C. mosaicus mosaicus. Their ecology and colour, in the context of von Lendenfeld's original descriptions, and the implications for fisheries are discussed.
SERENADES AND ELEGIES: THE RECENT MUSIC OF HUGH WOOD — PART II
Authors: Venn Edward
Published: June 2005

Geoffrey Hill's latest book of poems, Scenes from Comus, borrows its title from Wood's op. 6, and is dedicated to the composer for his seventieth birthday. The two men have been friends for many years and are exact contemporaries: for the poet's seventieth birthday, Wood wrote a vocal-instrumental setting of Hill's Tenebrae. This interchange between poet and musician highlights Wood's abiding concern with poets and poetry, and particularly English verse of the 20th century. He has described this repertoire as ‘a treasure-house, and our poets continue to produce good lyric poetry to this day: it's a waste of being English not to draw on these riches; and the composer has a particular duty to the poets of his own time’. More recently, Jeremy Thurlow has drawn attention to Wood's ‘idiomatic and refined response to English verse: his songs for voice and piano form a considerable part of his oeuvre and must be considered the most distinctive and substantial contribution to British song-writing since Britten and Tippet’.
Recent discs of Egon Wellesz
Authors: Conway Paul
Published: June 2005

EGON WELLESZ: Vorfrühling; Leben, Traum und Tod; Lied der Welt; Sonette der Elizabeth Barrett Browning; Ode an die Musik; Vision; Symphonischer Epilog. Regina Klepper (sop), Sophie Koch (mezzo), Deutsches Symphonie-Orchester Berlin, c. Roger Epple. Capriccio 67077.

WELLESZ: Symphonies Nos. 1 and 8; Symphonischer Epilog. Radio Symphonieorchester Wien, c. Gottfried Rabl. CPO 999998-2.

WELLESZ: Symphonies Nos. 2 and 9. Radio Symphonieorchester Wien, c. Gottfried Rabl. CPO 999997-2.

WELLESZ: Prosperos Beschwörungen; HONEGGER: Symphony No. 5; STRAVINSKY: Le Sacre du printemps; JANÁČEK: Sinfonietta; WEBERN: Passacaglia; Six Movements op. 6; BERG: Der Wein; SCHOENBERG: Pelleas und Melisande; SCHMIDT: Symphony No. 2. Vienna Philharmonic c. Haitink, Ansermet, Markevitch, Kubelik, Mehta, Dorov, Bohm, Leinsdorf. Andante AND4080.
Berlin: Hindemith's ‘Klaviermusik mit orchester’
Authors: Rickards Guy
Published: June 2005

Paul Wittgenstein's commissioning of concertos for piano left-hand is as enviable a legacy as any performer could wish to have, centred as it is on concertos by Korngold, Franz Schmidt (who also penned for Wittgenstein a set of Concertante Variations on a theme of Beethoven with orchestra and three piano quintets), Richard Strauss, Prokofiev, Britten (his op. 21 Diversions) and Ravel. Yet the maimed pianist's quixotic attitude to the works he received is almost as remarkable. Ravel he offended by the liberties he took with the solo part, while Prokofiev's Concerto No. 4 languished unplayed for a quarter of a century, until three years after the composer's death. Yet these cases pale into insignificance compared to the treatment meted out to the concerto that Paul Hindemith wrote for Wittgensein in 1923.
FIVE LONDON REPORTS
Authors: Miller Malcolm , Anderson Martin
Published: June 2005

Purcell Room: Charles Hart

Manoukian Cultural Centre, St Yegiche's Armenian Church, Cranley Gardens: Mikhail Pletnev

Purcell Room: Roxanna Panufnik

Leighton House and The Warehouse: Jonathan Powell, ‘Finland Piano Suomi’

Leighton House: Richard Dubugnon's ‘Mikroncerto III’
Central–Provincial Relations at the CCP Central Committees: Institutions, Measurement and Empirical Trends, 1978–2002
Authors: Sheng Yumin
Published: June 2005

What is the nature of central–provincial relations in China? How have they evolved during the era of reform and opening? How can we begin to measure and assess the dynamic changes empirically and consistently? This article tackles these questions by examining year-to-year changes in aggregate trends of provincial presence at the Communist Party Central Committee from 1978 to 2002. After first sketching its formal workings, it highlights how the centre is institutionally empowered to exert political leverage over the provinces at the Central Committee. Drawing upon a new dataset that differentiates among three types of Central Committee membership, it shows evidence of declining provincial shares in full Central Committee membership, a conventionally used indicator of provincial clout at the centre, but rising shares in its alternate membership and Politburo full membership. It concludes, on balance, that central political strength remains resilient in this period.
The Emergence of Greater China: The Economic Integration of Mainland China, Taiwan and Hong Kong. By Yung-Wing Sung. [Basingstoke and New York: Palgrave Macmillan, 2005. xvi+236 pp. ISBN 0-333-62599-4.]
Authors: Kueh Y. Y.
Published: June 2005

This is a highly readable book about the emerging economic complex of “Greater China.” The author, based at the Chinese University of Hong Kong, is the foremost authority on the subject matter. The book, which culminates from well over a decade of painstaking research and publication, traces the process and pattern of economic integration among the Chinese trio – the Chinese mainland, Hong Kong and Taiwan – over the past two decades or so. The analysis is set against the broader background of Chinese economic reforms and opening to the West, as well as the changing political context in East Asia that has facilitated increased economic interaction in the region.

The book starts with a broad description of the economic structure and relative economic strengths of the Chinese trio, and furnishes a useful conceptual framework for understanding the evolving economic relationships. Chapter two shows how FDI (foreign direct investment) from Hong Kong and Taiwan has triggered an accelerated process of integration with the mainland, and as a result led to the drastic expansion of China's external trade. Chapter three examines the particular characteristics of economic integration between Hong Kong and the mainland on the one hand, and between the two sides of the Taiwan Strait on the other hand. It reveals how cultural (affinity) and geographical (proximity) factors have played a role, and what policy readjustments have been made in the three constituent parts of the “China circle” to bring about a “new brand of ‘new-style’ economic integration,” which is unique in the global context of trade and investment liberalization.
China Along the Yellow River: Reflections on Rural Society. By Cao Jinqing, translated by Nicky Harman and Huang Ruhua. [London and New York: RoutledgeCurzon, 2005. 254 pp. $65.00. ISBN 0-415-34113-2.]
Authors: Wilson Scott
Published: June 2005

Periodically, Chinese leaders have ventured out on inspection tours directly to observe local conditions away from the capital. Cao Jinqing wrote this book on rural life in Henan as a daily log of conversations and observations, and it reads like the report of such an inspection tour. Along the tour, the author ruminates on rural development problems such as the rising tax and fee burden on farmers and the stagnation of agricultural growth that Henan faces. The author traces such problems to political, economic, and cultural institutions.

Cao Jinqing primarily focuses on xiang-level administration, and he advances three main points. First, he argues that China's administrative structure has created a set of opportunities for corruption and political pressure that have pushed an enormous financial burden onto farmers. Xiang officials face pressure to provide schools and other social services for their populations, yet education budgets alone can eclipse the statemandated cap of taxes and fees, set at five per cent of farmers' income. From above, local officials are pressed to expand their industrial base and agricultural production by investing in factories, converting grain fields to orchards, and implementing agricultural technology, all of which requires officials to extract fees. Such projects help cadres advance their careers, but they literally come at the expense of farmers. Total fees, according to one official, reach as high as 30–50 per cent of farmers' household income (p. 208). The political structure also creates incentives for local corruption. Several cadres interviewed suggest that corrupt officials get ahead and that upright officials are denied promotion.
Wild Grass: Three Stories of Change in Modern China. By Ian Johnson. [New York: Pantheon, 2004. ix+324 pp. $24.00. ISBN 0-375-42186-6.]
Authors: Blecher Marc
Published: June 2005

The willingness of ordinary Chinese to take extraordinary risks to challenge their state is widely known. Just what kind of people they are, and what wellsprings of personality and events drive them to do so, is harder to fathom. Ian Johnson's Wild Grass provides three fascinating cases that illuminate the question. Moreover, it implicitly points to the power of law not just to shape protest, but to bring it about in the first place. It's also a great read.

His first protagonist is Ma Wenlin, the “peasant champion.” A pretty ordinary 1962 Xi'an university graduate who worked quietly in local government for decades, in the early 1990s he taught himself the law and became a “legal worker,” concentrating mainly on contract and civil cases. In 1997, during a visit to his ancestral home, local farmers, inspired by a successful case nearby, prevailed on him to file a class-action suit against their township government for excessive levies. He demurred, but the farmers pressured him backhandedly by starting rumours that he was bribed by local officials to stay out of the matter. Rather than turning against them, he felt he had to clear his good name. He took the case. The failure of his legal filing, combined with his stubborn personality, his moral sensibility, and the inoculation against authority provided by the Cultural Revolution, emboldened him to participate in a political fight for his beleaguered clients. He helped the farmers organize local demonstrations, all citing the law, and eventually found his way to the Petition and Appeals Office of the State Council in Beijing.
The Great Wall of Confinement: The Chinese Prison Camp Through Contemporary Fiction and Reportage. By Philip F. Williams and Yenna Wu. [Berkeley, Los Angeles and London: University of California Press, 2004. xi+248 pp. $21.95; $55.00. ISBN 0-520-22779-4.]
Authors: Bakken Børge
Published: June 2005

By the “Great Wall of Confinement,” the authors refer to the prison camp system established by the Chinese Communist Party after 1949. The two crucial components of this system are the laogai system (laodong gaizao, translated in the book to “remolding through labour” rather than the more often used “reform through labour”), and the laojiao system (laodong jiaoyang) or “reeducation through labour.” Let me say at once that this book is much more than an analysis of the literature surrounding the phenomenon of the prison camps. Through memoirs from former inmates and reportage literature we learn many detailed facts about the Chinese camp system, details equally valuable to the legal and the social science scholar.

The book describes in detail the daily life of the camps, the prison conditions and the system's methods of arrest, detention, solitary confinement, torture for confessions, famine, degradation of prisoners, and a range of practices showing the security forces' discretionary powers and the “flexibilities” of informal sentencing. The authors emphasize both the modern ideology of remoulding and the traditional legalist (fajia) roots of a “very malleable sort of law.” Williams and Wu commendably combine a range of valuable empirical detail with a more general theoretical analysis of the historical, cultural and systemic roots and practices of the camp system.

The only exceptions to generally harsh conditions in the PRC camps were the special prisons for high-ranking persons like the famous Fushun prison in Liaoning province which contained the last Manchu emperor, Puyi, high-ranking prisoners of war such as former Kuomintang top military officers, and Japanese prisoners of war.
The Troublesome Legacy of Commissioner Lin: The Opium Trade and Opium Suppression in Fujian Province, 1820s to 1920s. By Joyce A. Madancy. [Harvard and London: Harvard East Asia Monograph, 2003. 430 pp. $50.00; £32.95. ISBN 0-674-01215-1.]
Authors: Zhou Xun
Published: June 2005

Visiting New York's Chinatown, it is surprising to find there a memorial statue of the legendary anti-opium hero, Lin Zexu, instead of the more usual statue of the father of modern China, Sun Yat-sen. Perhaps Lin deserves his place in New York's Chinatown: it is generally believed the history of Chinese migration into the New World was a chapter of humiliation, resulting from the evil opium and the opium trade. Until very recently, the conventional wisdom has been that it was the opium trade that ended the house of Qing, and that opium had turned China into a nation of hopeless addicts, smoking themselves to death while their civilization descended into chaos (a view challenged by Dikötter, Laaman and Zhou in Narcotic Culture: A History of Drugs in China).

In her book The Troublesome Legacy of Commissoner Lin, Joyce Madancy argues that, like opium, Lin Zexu was turned into a potent symbol of nascent Chinese nationalism (p. 5). Like opium, the legacy of Lin continued well into the 20th century. In his native Fujian, for instance, Lin “came to represent the vitality of elite activism and the complex links between provincial, national, and international interests. Lin Zexu's character and mission embodied the themes and motivations of Fujian's late Qing opium reformers – the righteousness of opium reform, pride in country and province, and a none-too-subtle slap at foreign imperialist greed.” Accordingly, during the late Qing/early Republican anti-opium campaign in Fujian, “reformist elites, and officials presided over the apotheosis of Lin Zexu, whose image loomed, literally and figuratively, over their efforts and shaped the rhetoric and tone of suppression” (p. 5).
In Search of an Identity: The Politics of History as a School Subject in Hong Kong, 1960s–2002. By Edward Vickers. [New York and London: Routledge, 2003. vii+321 pp. £60.00. ISBN 0-415-94502-X.]
Authors: Jones Alisa
Published: June 2005

In recent years, much research has been published on nationalism and national identity formation in East Asia. It has been frequently noted that “official” historical narratives disseminated through school curricula have been crucial to popularizing state-sanctioned national and worldviews and legitimizing the polity. Yet, excepting research into the international controversies surrounding Japanese history textbook portrayals of Japan's wartime past, few studies have looked beyond a handful of government directives and textbooks. What has been written, moreover, has often assumed that political authoritarianism and/or the highly centralized nature of curriculum and textbook development in most of East Asia (at least until very recently) have rendered history education little more than a top-down process of attempted ideological indoctrination, an assumption reinforced by theories that depict mass education primarily as a means by which social and political elites sustain their hegemony.

In Search of an Identity offers a long overdue examination of this neglected field, focusing on the interesting case of Hong Kong where the territory's dual Chinese–British heritage has been reflected in the inclusion of two wholly separate history subjects in the school curriculum: Chinese history, taught in Cantonese and chronologically narrating 5,000 years of Chinese civilization; and history, taught predominantly in English and covering assorted historical periods and events world-wide. This meticulously detailed study charts the evolution of the history subject at fourth to sixth-form levels (ages 14–18) from the late 1960s through retrocession up to 2002, coverage which is extended in the forthcoming paperback edition to 2004 to include the latest textbook revisions (Comparative Education Research Centre, Hong Kong University, 2005).
Hitchcock with a Chinese Face: Cinematic Doubles, Oedipal Triangles, and China's Moral Voice (with DVD). By Jerome Silbergeld. [Seattle: University of Washington Press, 2004. 160 pp. £22.95. ISBN 0-295-98417-1.]
Authors: Berry Chris
Published: June 2005

Jerome Silbergeld introduced an art history approach into Chinese film studies with China into Film: Frames of Reference in Contemporary Chinese Cinema in 2000. Hitchcock with a Chinese Face goes further. Like an art historian selecting three seemingly disparate paintings and demonstrating their links, Silbergeld chooses a film each from Hong Kong, Taiwan and mainland China, but argues that they pursue similar aesthetic and political directions. The result is a virtuoso display of intense textual and inter-textual exegesis, informed by an in-depth knowledge of the pre-modern Chinese arts, contemporary Chinese political culture, and globally circulated Western culture (including Hitchcock). It is also a challenge to the discipline of film studies itself.

The three films Silbergeld selects for analysis are Lou Ye's 2000 film from mainland China, Suzhou River (Suzhou he); Yim Ho's 1994 Hong Kong film, The Day the Sun Turned Cold (Tianguo nizi); and the final part of Hou Hsiao Hsien's 1995 Taiwan trilogy, Good Men, Good Women (Hao nan, hao nü,). He acknowledges that the project began as a personal indulgence allowing him to explore further some of his favourite films. However, his engagement with the films leads him to argue that each one, in its own way, deconstructs the commonly circulated idea of a unified Chinese culture, engages powerfully with morality, is narratively complex and anti-commercial, mobilizes a cosmopolitan knowledge of world cinema, and displays an unusual degree of interest in individual psychology and oedipality. The latter elements help to ground the comparisons to Hitchcock (as well as to Hamlet, Dostoevsky, Faulkner and others).
Encyclopedia of Contemporary Chinese Culture. Edited by Edward L. Davis. [London and New York: Routledge, 2005. xxiv+786 pp. $210.00. ISBN 0-415-24129-4.]
Authors: Brown Jeremy
Published: June 2005

Readers seeking information about prominent urban Chinese artists, writers, composers, film-makers, public intellectuals, and socio-cultural trends in the reform period will find much of use in the Encyclopedia of Contemporary Chinese Culture, a collaborative transnational effort that is unfortunately marred by unevenness and sloppy editing. Browsers will also find lively and opinionated essays about cars and taxis, falun gong, democracy, dating and sex shops.

Editor Edward L. Davis gave free reign to the contributors of the almost 1,200 entries in this fifth volume, encouraging them to pass judgment and editorialize. He also wisely involved mainland scholars like Yue Daiyun and Dai Jinhua when drawing up the lists of entries, and called upon Francesca Dal Lago to oversee the book's excellent sections on visual arts. While the Encyclopedia's list of contributors includes prominent, well-established scholars (Timothy Cheek on intellectuals and academics, Frank Dikötter on prisons, and Geremie Barme´ on seemingly anything he wanted to write about), its large number of young, Chineseborn scholars based in North America and Europe reflects an important shift in the field of Chinese studies.

Entries, varying in length from a single paragraph to ten pages (see Lionel Jensen's piece on falun gong, for example), are organized alphabetically, include cross-references, and are often followed by suggestions for further reading. A helpful thematic classified entry list precedes the entries themselves. Unfortunately, problematic organization undermines the book's usefulness for both literate Chinese readers and those with no knowledge of the language. Pinyin renderings of names and phrases are not accompanied by Chinese characters, hampering the task of scholars hoping to conduct further primary-source research on a particular person.