bluuebunny/crossref_metadata_2025_split huggingface dataset:
The Son‐Rise Program1 intervention for autism: an investigation into family experiences
Authors: Williams K. R. , Wishart J. G. 
Published: May 2003

AbstractBackground  Despite the increasing involvement of parents as therapists in interventions for their children with autism, research to date has focused almost exclusively on the outcome for the child, and little is known about the effects of involvement on the whole family. This is true even of highly intensive home‐based approaches such as the Son‐Rise Program (SRP), the focus of the present paper. A longitudinal questionnaire‐based study is reported which investigated a number of potential positive and negative effects for the family, how these changed over time, and their relation to child characteristics and patterns of intervention implementation.Methods  Questionnaires examining family demographics, patterns of intervention use and perceived family effects were distributed three times over the course of a year to families who had attended an initial training course in the use of the SRP.Results  The results indicated that, although involvement led to more drawbacks than benefits for the families over time, family stress levels did not rise in all cases. Few relationships were found between family effects and patterns of intervention use, although there was a strong connection with parental perceptions of intervention efficacy.Conclusions  The findings of the present study emphasize the need for those supporting families using home‐based interventions to consider the needs of the whole family. This may be especially important if there are periods during which the family find the intervention to be less effective. Families embarking on such intensive approaches may also benefit from considering ways in which any disruption to family life can be minimized.
The role of coping in maintaining the psychological well‐being of mothers of adults with intellectual disability and mental illness
Authors: Kim H. W. , Greenberg J. S. , Seltzer M. M. , Krauss M. W. 
Published: May 2003

AbstractBackground  Mothers who have a child with intellectual disability (ID) or mental illness face a lifetime of caregiving responsibilities and challenges. The present study investigated changes over time in how mothers cope with the challenges of caring for an adult child with disabilities and the effects of changes in coping on maternal well‐being.Methods  A sample of 246 ageing mothers of adults with ID and 74 mothers of adults with mental illness was drawn from two parallel longitudinal studies of later‐life caregiving.Results  There was considerable variability at the individual level in the degree to which mothers changed over time in their use of problem‐focused and emotion‐focused coping strategies. For both groups, an increase in their use of emotion‐focused coping led to declining levels of well‐being. For the parents of adults with ID, an increase in their use of problem‐focused coping resulted in a reduction in distress and an improvement in the quality of the relationship with their adult child. For the parents of adults with mental illness, an increase in the use of problem‐focused coping had no effect on levels of distress, but led to an improved relationship with their adult child.Conclusions  The present study underscores the importance of coping in the lives of older mothers of adults with disabilities.
Supporting families over the life course: mapping temporality
Authors: Grant G. , Nolan M. , Keady J. 
Published: May 2003

AbstractBackground  The present paper addresses a rather neglected dimension of family caregiving, its temporality. Many accounts of caregiving assume a state of stasis, and therefore, overlook factors which shape the evolving experience of family caregiving over the life course.Methods  The paper begins by offering some reflections on theoretical and methodological issues identified by life‐course researchers.Results  Based both on theoretical propositions and a growing body of empirical evidence, this paper offers a heuristic for thinking about caregiving stages applied to families supporting people with intellectual disability.Conclusions  This heuristic is used to suggest further avenues of research and development.
Toward assessing family outcomes of service delivery: validation of a family quality of life survey
Authors: Park J. , Hoffman L. , Marquis J. , Turnbull A. P. , Poston D. , Mannan H. , Wang M. , Nelson L. L. 
Published: May 2003

AbstractBackground  The concept of family quality of life (QoL) has emerged as an important outcome of service delivery for individuals with disabilities and their families. The present study describes the process of developing a tool to measure family QoL.Methods and Results  A total of 1197 respondents participated in a national field test. Through factor analysis, the survey was refined in several ways: (1) the preliminary 10‐domain structure was reduced to a five‐domain structure; (2) a total of 41 items were selected for the revised survey; and (3) wordings were clarified.Conclusions  The implications for future research and practice are discussed.
Mothers of children and adolescents with intellectual disability: social and economic situation, mental health status, and the self‐assessed social and psychological impact of the child's difficulties
Authors: Emerson E. 
Published: May 2003

AbstractBackgroundFew large‐scale studies with well‐constructed samples have compared the socio‐economic circumstances and social impact of raising a child with intellectual disability (ID). The aims of the present paper were to: (1) compare the socio‐economic situation of mothers raising a child with ID to that of mothers of non‐ID children; (2) assess the contribution of raising a child with ID to negative psychological outcomes for mothers; and (3) identify variables associated with negative psychological outcomes among mothers of children with ID.MethodsThe 1999 Office for National Statistics survey, Mental Health of Children and Adolescents in Great Britain, 1999, collected information on a multistage stratified random sample of 10 438 children between 5 and 15 years of age across 475 postal code sectors in England, Scotland and Wales. Secondary analysis was undertaken of the social and economic circumstances, and stress reported by 245 mothers of sampled children with ID and a comparison group of 9 481 mothers of sampled children who did not have ID.Results The results indicate that:(1) families supporting a child with ID were significantly economically disadvantaged when compared with families supporting a child who did not have ID; (2) when compared with mothers of sampled children who did not have ID, mothers of sampled children with ID reported that their child's difficulties resulted in greater social and psychological impact; (3) having a child with ID marginally reduced the odds of mothers screening positive for having mental health problems (once all other variables were taken into account); and (4) among mothers of children with ID, mental health problems were associated with the child's difficulties having a greater social impact, having a boy, the child experiencing more than one potentially stressful life event, poverty, receipt of means‐tested welfare benefits and ‘unhealthy’ family functioning.ConclusionsThese results highlight the importance of combating poverty among children with ID and their families, and the need to develop more complex models of understanding and intervention.
The intermembrane space of plant mitochondria contains a DNase activity that may be involved in programmed cell death
Authors: Balk Janneke , Chew Su Kit , Leaver Christopher J. , McCabe Paul F. 
Published: May 2003

SummaryThe key role for mitochondria in mammalian apoptosis, a form of programmed cell death (PCD), is well established, but a similar role for plant mitochondria is just emerging. In order to unravel the molecular mechanisms linking plant mitochondria to the downstream events of PCD, we have developed an Arabidopsis cell‐free system that can be used to monitor biochemical and morphological changes in isolated nuclei that are associated with PCD. Using this system, two activities that resulted in nuclear DNA degradation could be distinguished, both of which were facilitated by the addition of mitochondria. One activity mediated the generation of 30 kb DNA fragments within 3 h and chromatin condensation within 6 h, when nuclei were incubated with mitochondria alone. The second activity required cytosolic extract in addition to mitochondria and resulted in oligonucleosome‐sized DNA cleavage after >12 h. Submitochondrial fractionation and pharmacological studies suggested the presence of an Mg2+‐dependent nuclease activity in the intermembrane space, which is responsible for the former in vitro activity. The evolutionary conservation of the role of mitochondria in PCD in animals and plants is discussed.
Leucine‐rich repeat‐mediated intramolecular interactions in nematode recognition and cell death signaling by the tomato resistance protein Mi
Authors: Hwang Chin‐Feng , Williamson Valerie M. 
Published: May 2003

SummaryThe root‐knot nematode resistance gene Mi from tomato encodes a nucleotide‐binding/leucine‐rich repeat (NB/LRR) protein with a novel amino‐terminal domain compared to related disease‐resistance genes. The closely linked paralog Mi‐1.1, which does not confer nematode resistance, encodes a protein 91% identical to the functional copy, Mi‐1.2. The chimeric construct Mi‐DS3, which encodes the 161 amino‐terminal residues from Mi‐1.1 fused to the remainder of Mi‐1.2, induces localized necrosis when transiently expressed in Nicotiana benthamiana leaves. We produced mutant constructs that exchanged sequences encoding each of the 40 amino acid differences from the Mi‐1.1 LRR region into Mi‐DS3 and into Mi‐1.2. For 23 of the substitutions, necrosis was lost upon transient expression of the mutated Mi‐DS3 in N. benthamiana, and nematode resistance was lost when the altered Mi‐1.2 was expressed in the tomato roots. One substitution, R961D, failed to give Mi‐DS3‐induced necrosis, but produced a dominant lethal phenotype when introduced into Mi‐1.2. This gain‐of‐function phenotype was suppressed by co‐expression with the amino‐terminal region of Mi‐1.1, suggesting that residue 961 is critical for negative regulation by the corresponding N‐terminal region. Substitutions of Mi‐1.1 residues 984–986 retained the ability to cause necrosis in Mi‐DS3, but resulted in loss‐of‐nematode resistance in Mi‐1.2, suggesting that these residues are essential for nematode recognition. None of the loss‐of‐function mutations in Mi‐1.2 had a dominant negative phenotype. These results indicate that the Mi‐1.2 LRR is involved in regulation of the transmission of the resistance response as well as in recognition of the nematode.
Two cis‐acting elements necessary and sufficient for gibberellin‐upregulated proteinase expression in rice seeds
Authors: Sutoh Keita , Yamauchi Daisuke 
Published: May 2003

SummaryIn germinating rice seeds, a cysteine proteinase (REP‐1), synthesized in aleurone‐layer cells, is a key enzyme in the degradation of the major storage protein, glutelin. The expression of the gene for REP‐1 (Rep1) is induced by gibberellins (GAs) and repressed by abscisic acid (ABA). To identify GA‐responsive elements in the Rep1 promoter, we developed a transient expression system in rice aleurone cells. Deletion and point‐mutation analyses indicated that the GA‐response complex was composed of TAACAGA, TAACGTA, and two copies of CAACTC. The two former sequences were identical to GAREs conserved in the promoter of genes for α‐amylase and proteinases in cereals. The latter, termed as CAACTC regulatory elements (CAREs), were novel GAREs. Gain‐of‐function experiments revealed that two pairs of GARE and CARE were necessary and sufficient to confer GA inducibility. The sequences were also required for effective transactivation by the transcription factor OsGAMyb. Four copies of either GARE or CARE showed transactivation neither by OsGAMyb nor by GA induction. CARE and GARE were also found in the promoters of a rice α‐amylase gene, RAmy1A, and a barley proteinase gene, EPB1, which are expressed in germinating seeds. Mutations of CARE in their promoters caused a loss of GA inducibility and GAMyb transactivation, suggesting that CARE is the regulatory element for GA‐inducible expression of hydrolase genes in the germinating seeds.
Characterization of Arabidopsis thaliana methyl‐CpG‐binding domain (MBD) proteins
Authors: Zemach Assaf , Grafi Gideon 
Published: May 2003

SummaryCytosine methylation at symmetrical CpG and CpNpG sequences plays a key role in the epigenetic control of plant growth and development; yet, the way by which the methylation signal is interpreted into a functional state has not been elucidated. In animals, the methylation signal is recognized by methyl‐CpG‐binding domain (MBD) proteins that specifically bind methylated CpG dinucleotides. In Arabidopsis thaliana, 12 putative MBD proteins were identified and classified into seven subclasses. Here, we characterized six MBD proteins representing four subclasses (II, III, IV, and VI) of the Arabidopsis MBD family. We found that AtMBD7 (subclass VI), a unique protein containing a double MBD motif, as well as AtMBD5 and AtMBD6 (subclass IV), bind specifically symmetrically methylated CpG sites. The MBD motif derived from AtMBD6, but not from AtMBD2, was sufficient for binding methylated CpG dinucleotides. AtMBD6 precipitated histone deacetylase (HDAC) activity from the leaf nuclear extract. The examined AtMBD proteins neither bound methylated CpNpG sequences nor did they display DNA demethylase activity. Our results suggest that AtMBD5, AtMBD6, and AtMBD7 are likely to function in Arabidopsis plants as mediators of the CpG methylation, linking DNA methylation‐induced gene silencing with histone deacetylation.
Fungal responsive fatty acid acetylenases occur widely in evolutionarily distant plant families
Authors: Cahoon Edgar B. , Schnurr Judy A. , Huffman Errol A. , Minto Robert E. 
Published: May 2003

SummaryThe fungal elicitor‐induced ELI12 gene from parsley has been previously shown to encode a divergent form of the Δ12‐oleic acid desaturase. In this report, we show that the ELI12 gene product is a fatty acid acetylenase or a triple‐bond‐forming enzyme. Expression of this enzyme in transgenic soybean seeds was accompanied by the accumulation of the Δ12‐acetylenic fatty acids, crepenynic and dehydrocrepenynic acids. Using PCR with degenerate oligonucleotides, we also show that homologs of the ELI12 gene are present in other members of the Apiaceae family. In addition, cDNAs for divergent forms of the Δ12‐oleic acid desaturase were detected among the expressed sequence tags (ESTs) from English ivy, an Araliaceae species, and sunflower, an Asteraceae species. As with the ELI12 gene, expression of these cDNAs in transgenic soybean embryos was accompanied by the accumulation of crepenynic and dehydrocrepenynic acids. Homologs of the sunflower acetylenase gene were also detected in other Asteraceae species, as revealed by PCR analysis of isolated genomic DNA. Results from Northern blot and EST analyses indicated that the expression of the sunflower gene, like ELI12, was induced by fungal elicitation. Overall, these results demonstrate that expressed genes for Δ12‐fatty acid acetylenases occur in at least three plant families, and are responsive to fungal pathogenesis. Natural products derived from crepenynic and dehydrocrepenynic acids that display antifungal, insecticidal, and nematicidal properties are distributed through at least 15 plant families. The acetylenases described here provide probes for chemotaxonomists, and facilitate functional genomic and molecular investigations of these defensive mechanisms.
Dominant repression of target genes by chimeric repressors that include the EAR motif, a repression domain, in Arabidopsis
Authors: Hiratsu Keiichiro , Matsui Kyoko , Koyama Tomotsugu , Ohme‐Takagi Masaru 
Published: May 2003

SummaryThe redundancy of genes for plant transcription factors often interferes with efforts to identify the biologic functions of such factors. We show here that four different transcription factors fused to the EAR motif, a repression domain of only 12 amino acids, act as dominant repressors in transgenic Arabidopsis and suppress the expression of specific target genes, even in the presence of the redundant transcription factors, with resultant dominant loss‐of‐function phenotypes. Chimeric EIN3, CUC1, PAP1, and AtMYB23 repressors that included the EAR motif dominantly suppressed the expression of their target genes and caused insensitivity to ethylene, cup‐shaped cotyledons, reduction in the accumulation of anthocyanin, and absence of trichomes, respectively. This chimeric repressor silencing technology (CRES‐T), exploiting the EAR‐motif repression domain, is simple and effective and can overcome genetic redundancy. Thus, it should be useful not only for the rapid analysis of the functions of redundant plant transcription factors but also for the manipulation of plant traits via the suppression of gene expression that is regulated by specific transcription factors.
AtNRAMP3, a multispecific vacuolar metal transporter involved in plant responses to iron deficiency
Authors: Thomine Sébastien , Lelièvre Françoise , Debarbieux Elise , Schroeder Julian I. , Barbier‐Brygoo Hélène 
Published: May 2003

SummaryMetal homeostasis is critical for the survival of living organisms, and metal transporters play central roles in maintaining metal homeostasis in the living cells. We have investigated the function of a metal transporter of the NRAMP family, AtNRAMP3, in Arabidopsis thaliana. A previous study showed that AtNRAMP3 expression is upregulated by iron (Fe) starvation and that AtNRAMP3 protein can transport Fe. In the present study, we used AtNRAMP3 promoter β‐glucoronidase (GUS) fusions to show that AtNRAMP3 is expressed in the vascular bundles of roots, stems, and leaves under Fe‐sufficient conditions. This suggests a function in long‐distance metal transport within the plant. Under Fe‐starvation conditions, the GUS activity driven by the AtNRAMP3 promoter is upregulated without any change in the expression pattern. We analyze the impact of AtNRAMP3 disruption and overexpression on metal accumulation in plants. Under Fe‐sufficient conditions, AtNRAMP3 overexpression or disruption does not lead to any change in the plant metal content. Upon Fe starvation, AtNRAMP3 disruption leads to increased accumulation of manganese (Mn) and zinc (Zn) in the roots, whereas AtNRAMP3 overexpression downregulates Mn accumulation. In addition, overexpression of AtNRAMP3 downregulates the expression of the primary Fe uptake transporter IRT1 and of the root ferric chelate reductase FRO2. Expression of AtNRAMP3::GFP fusion protein in onion cells or Arabidopsis protoplasts shows that AtNRAMP3 protein localizes to the vacuolar membrane. To account for the results presented, we propose that AtNRAMP3 influences metal accumulation and IRT1 and FRO2 gene expression by mobilizing vacuolar metal pools to the cytosol.
Enhanced ethylene responsiveness in the Arabidopsis eer1 mutant results from a loss‐of‐function mutation in the protein phosphatase 2A A regulatory subunit, RCN1
Authors: Larsen Paul Brian , Cancel Jesse Daniel 
Published: May 2003

SummaryEthylene signaling in Arabidopsis begins with a family of five ethylene receptors that regulate the activity of the Raf‐like kinase, CTR1. Recent work to identify novel factors required for modulating ethylene signaling resulted in the isolation of enhanced ethylene response 1 (eer1), a mutant that displays both increased sensitivity and increased amplitude of response to ethylene. Molecular cloning of eer1 reveals that its mutant phenotype results from a loss‐of‐function mutation in the previously characterized RCN1, one of three PP2A A regulatory subunits in Arabidopsis. Our analysis shows that neither RCN1 expression nor PP2A activity is regulated by ethylene. Instead, we found that Arabidopsis PP2A‐1C, a PP2A catalytic subunit previously characterized as interacting with RCN1, associates strongly with the kinase domain of CTR1 in vitro. This likely represents a role for PP2A in modulation of CTR1 activity because an in vitro kinase assay did not reveal phosphorylation of either RCN1 or PP2A‐1C by CTR1, indicating that neither of them is a substrate for CTR1. PP2A activity is required for Ras‐dependent activation of mammalian Raf, with reductions in PP2A activity significantly compromising the effectiveness of this mechanism. Our genetic and biochemical results suggest that a similar requirement for PP2A activity exists for ethylene signaling, with loss‐of‐function mutations affecting PP2A activity possibly reducing the effectiveness of CTR1 activation, thus lowering the threshold required for manifestation of ethylene response.
Functional analysis of the 37 kDa inner envelope membrane polypeptide in chloroplast biogenesis using a Ds‐tagged Arabidopsis pale‐green mutant
Authors: Motohashi Reiko , Ito Takuya , Kobayashi Masatomo , Taji Teruaki , Nagata Noriko , Asami Tadao , Yoshida Shigeo , Yamaguchi‐Shinozaki Kazuko , Shinozaki Kazuo 
Published: May 2003

SummaryTo study the functions of the nuclear genes involved in chloroplast development, we systematically analyzed albino and pale‐green Arabidopsis thaliana mutants by using a two‐component transposon system based on the Ac/Ds element of maize as a mutagen. One of the pale‐green mutants, albino or pale green mutant 1 (designated as apg1), did not survive beyond the seedling stage, when germinated on soil. The chloroplasts of the apg1 plants contained decreased numbers of lamellae with reduced levels of chlorophyll. A gene encoding a 37 kDa polypeptide precursor of the chloroplast inner envelope membrane was disrupted by insertion of the Ds transposon in apg1. The 37 kDa protein had partial sequence similarity to the S‐adenosylmethionine‐dependent methyltransferase. The apg1 plants lacked plastoquinone (PQ), suggesting that the APG1 protein is involved in the methylation step of PQ biosynthesis, which is localized at the envelope membrane. Our results demonstrate the importance of the 37 kDa protein of the chloroplast inner envelope membrane for chloroplast development in Arabidopsis.
The first example of anti‐Diegob found in a Polish woman with the Di(a+b–) phenotype and haemolytic disease of the newborn not requiring treatment
Authors: Lenkiewicz B. , Żupańska B. 
Published: May 2003

Summary.  All pregnant women with anti‐Diegob (anti‐Dib) described so far were non‐Caucasians. We present the case of a Polish Di(a+b–) woman with anti‐Dib, which did not bind complement, was immunoglobulin G3 (IgG3) alone and had very low functional activity. She delivered a Di(a+b+) infant with a positive direct antiglobulin test and the antibody in his serum but very mild haemolytic disease.Both parents of the pregnant woman were Di(a+b+), so were all her three children. The whole family have been living in a small village in southeastern Poland for a long time. The rare Diego phenotypes, found now and previously in Poland, suggest gene admixture introduced as a result of Poland being invaded by Mongolian‐background Tatars during the past centuries.