123,598 research outputs found
Abitare la condivisione. Un confronto divulgativo sul tema dell'abitare e della condivisione
No full textIl volume è l’ultimo risultato della ricerca “Cohousing. Programmi e progetti per la riqualificazione del patrimonio esistente” svoltasi nel Dipartimento di Architettura dell’Università degli Studi Roma Tre e delle riflessioni scaturite nel Laboratorio di Laurea
“Spazi dell’abitare” svoltosi nello stesso Dipartimento negli anni accademici 2015-16 e 2016-17. “Abitare la condivisione” è quindi il risultato di un confronto divulgativo tenuto da studiosi e professionisti sul tema dell’abitare e della condivisione
Cohousing. Programmi e progetti per la riqualificazione del patrimonio esistente. Cohousing. Programs and projects to recover heritage buildings
No full textThis book is the upshot of the Study Day entitled “Cohousing. Programs and projects to recover heritage buildings” staged in Rome on 24 October 2014, and organized by a team of researchers (Adolfo F. L. Baratta, Fabrizio Finucci, Stefano Gabriele, Annalisa Metta, Luca Montuori, Valerio Palmieri) of the Department of Architecture of the Roma Tre University, with a multi-disciplinary approach. The book contains the contributions of scholars, professionals and voluntary sector operators relating to two topics all set to play a lead role in global policies in the next decade: the topic of houses, which for years in Italy has been addressed with a welfare rather than structural approach, and the topic of the requalification of the existing building heritage, which must be recovered in terms of architectural and urban quality, safety, efficiency and inclusiveness. “Cohousing. Programs and projects to recover heritage buildings” is therefore the upshot of an instructive discussion on the topic of sharing common spaces, more widespread in the north-European countries, combined with the topic of the requalification of existing buildings, more central to the Mediterranean area.This book is the upshot of the Study Day entitled “Cohousing. Programs and projects to recover heritage buildings” staged in Rome on 24 October 2014, and organized by a team of researchers (Adolfo F. L. Baratta, Fabrizio Finucci, Stefano Gabriele, Annalisa Metta, Luca Montuori, Valerio Palmieri) of the Department of Architecture of the Roma Tre University, with a multi-disciplinary approach. The book contains the contributions of scholars, professionals and voluntary sector operators relating to two topics all set to play a lead role in global policies in the next decade: the topic of houses, which for years in Italy has been addressed with a welfare rather than structural approach, and the topic of the requalification of the existing building heritage, which must be recovered in terms of architectural and urban quality, safety, efficiency and inclusiveness. “Cohousing. Programs and projects to recover heritage buildings” is therefore the upshot of an instructive discussion on the topic of sharing common spaces, more widespread in the north-European countries, combined with the topic of the requalification of existing buildings, more central to the Mediterranean area
A MOLECULAR EXPLANATION OF SLC25A1 DEFICIENCY RESULTING IN AGENESIS OF CORPUS CALLOSUM AND OPTIC NERVE HYPOPLASIA
No full textMitochondrial carriers (MCs) form a large family of nuclear-encoded transporters embedded in the inner mitochondrial membrane and in a few cases in other organelle membranes (Palmieri, 2013). The members of this superfamily are widespread in eukaryotes and involved in numerous metabolic pathways and cell functions. They can be easily recognized by their striking sequence features, i.e., a tripartite structure, six transmembrane α-helices and a 3-fold repeated signature motifs. Members of the family vary greatly in the nature and size of their transported substrates, modes of transport (i.e., uniport, symport or antiport) and driving forces, although the molecular mechanism of substrate translocation may be basically the same. In recent years mutations in the MC genes have been shown to be responsible for 11 diseases (Palmieri, 2013), highlighting the important role of MCs in metabolism. MC impairing mutations affect three main regions crucial for substrate translocation. A first group of mutations affects MC conformational changes and locates at PG levels or at the aromatic belts (Pierri et al., 2013). A second group of mutations affects substrate specificity and locates at the common substrate binding site (Robinson et al., 2008) and at the substrate binding area (Pierri et al., 2013). A further group of mutations locate at residues of the m-/c-gates (Palmieri et al., 2013; Robinson et al., 2008) and at residues of the m-gate area (Pierri et al. 2013). For this last group of mutations, it appears difficult to establish if the impaired function is due to the lack of substrate specificity (or substrate recognition) or to the wrong triggering of conformational changes. Two mutations, one at the PG level 1 and one at the common substrate binding site, impairing citrate translocation within SLC25A1_CTP protein are presented. The two mutations are found to be responsible of agenesis of corpus callosum and optic nerve hypoplasia (Edvardson et al., 2013).
References
1. Palmieri F. The mitochondrial transporter family SLC25: identification, properties and physiopathology. Mol Aspects Med. 2013;34:465.
2. Pierri CL, Palmieri F, De Grassi A. Single-nucleotide evolution quantifies the importance of each site along the structure of mitochondrial carriers. Cell Mol Life Sci. 2013.
3. Robinson AJ, Overy C, Kunji ER. The mechanism of transport by mitochondrial carriers based on analysis of symmetry. Proc Natl Acad Sci U S A. 2008;105:17766.
4. Edvardson S, Porcelli V, Jalas C, Soiferman D, Kellner Y, Shaag A, Korman SH, Pierri CL, Scarcia P, Fraenkel ND, Segel R, Schechter A, Frumkin A, Pines O, Saada A, Palmieri L, Elpeleg O. Agenesis of corpus callosum and optic nerve hypoplasia due to mutations in SLC25A1 encoding the mitochondrial citrate transporter. J Med Genet. 2013;50:240
Mitochondrial carriers and related diseases
No full textSince the end of nineties numerous mitochondrial diseases have been found to be related to mutations in nuclear genes encoding mitochondrial carriers, a family of proteins that shuttle a variety of metabolites across the mitochondrial membrane. To date eleven disorders are known to be caused by defects of mitochondrial carriers. Mutations of mitochondrial carrier genes are responsible for carnitine/acylcarnitine carrier deficiency, ornithine carrier deficiency (HHH syndrome), aspartate/glutamate isoform 1 deficiency (global cerebral hypomyelination), aspartate/glutamate isoform 2 deficiency (CTLN2 and NICCD), amish microcephaly, neonatal myoclonic epilepsy, congenital sideroblastic anemia, PiC deficiency, ADP/ATP carrier isoform 1 deficiency and involved in neuropathy and bilateral striatal necrosis and adPEO (autosomal dominant progressive external ophthalmoplegia). We propose un updated overview of these diseases. We shall also discuss the role of missense mutations in impairing mitochondrial carrier function and the consequent severe damage to the mitochondrial matrix supply with substrates destined to specific metabolic pathways. Despite the substantial progress that has been made in our understanding of the molecular bases of mitochondrial carrier associated diseases, specific pharmacological therapies are not yet available. Current therapies are symptomatic and usually based on specific dietary measures. New therapeutic approaches are under investigation for some of these diseases.
For further reading
Palmieri F. (2008) Diseases caused by defects of mitochondrial carriers: a review. Biochim Biophys Acta; 1777:564-78.
Palmieri F, Pierri CL (2010) Structure and function of mitochondrial carriers - Role of the transmembrane helix P and G residues in the gating and transport mechanism. FEBS Lett. 584:1931-9.
Tessa A, Fiermonte G, Dionisi-Vici C, Paradies E, Baumgartner MR, Chien YH,Loguercio C, de Baulny HO, Nassogne MC, Schiff M, Deodato F, Parenti G, Rutledge SL, Vilaseca MA, Melone MA, Scarano G, Aldamiz-Echevarría L, Besley G, Walter J, Martinez-Hernandez E, Hernandez JM, Pierri CL, Palmieri F, Santorelli FM. (2009) Identification of novel mutations in the SLC25A15 gene in hyperornithinemia-hyperammonemia-homocitrullinuria (HHH) syndrome: a clinical, molecular, and functional study. Human Mutation; 30:741-8.
Wibom R, Lasorsa FM, Töhönen V, Barbaro M, Sterky FH, Kucinski T, Naess K, Jonsson M, Pierri CL, Palmieri F, Wedell A. (2009) AGC1 deficiency associated with global cerebral hypomyelination. N Engl J Med.; 361:489-95.
Iacobazzi V, Convertini P, Infantino V, Scarcia P, Todisco S, Palmieri F. (2009) Statins, fibrates and retinoic acid upregulate mitochondrial acylcarinitine carrier gene expression. Biochem Biophys Res Commun.; 388:643-7
Uniformità ed obiettività nell’inquadramento medico-legale del danno psichico: una proposta metodologica (in collab. Con V. Secchione e L. Palmieri) Med. Leg. Quad. Cam., , 3, 2001.
No full textUniformità ed obiettività nell’inquadramento medico-legale del danno psichico: una proposta metodologic
Arabidopsis thaliana Mitochondrial ATP-Mg/Phosphate Carriers: Biochemical Characterization and Organ Expression
No full textMembers of the mitochondrial carrier family transport metabolites, nucleotides and cofactors across the inner membrane of mitochondria. Substrates for about half of the 58 family members in Arabidopsis thaliana have been identified (1). Here, we report the functional characterization and organ distribution for three of them, named AtAPC1, AtAPC2 and AtAPC3, that exhibit high sequence similarities with the human mitochondrial ATP-Mg/phosphate carriers (APC) (2).
The expression levels of the AtAPCs in various tissues were analyzed by quantitative real-time PCR and the beta-glucuronidase (GUS) reporter system. Recombinant AtAPCs were expressed in Escherichia coli, purified and reconstituted into liposomes that were used in transport assays (3).
Under normal physiological conditions AtAPC1 was expressed at least five times more than the other two isoforms in flower, leaf, stem, root and seedlings. However, in stress conditions the expression levels of AtAPC1 and AtAPC3 change. The transport assays showed that all three AtAPCs transport phosphate, AMP, ADP, ATP, adenosine 5'-phosphosulfate and, to a lesser extent, other nucleotides. AtAPC2 and AtAPC3 also have the ability to transport sulfate and thiosulfate. The three AtAPCs catalyzed a counter-exchange transport that was saturable and inhibited by pyridoxal-5'-phosphate. The transport activities of the AtAPCs were also inhibited by the addition of EDTA or EGTA and stimulated by the addition of Ca2+.
Given that phosphate and sulfate can be recycled via their own specific carriers, these findings indicate that AtAPCs can catalyze net transfer of adenine nucleotides across the inner mitochondrial membrane in exchange for phosphate (or sulfate), and that this transport is regulated both at the transcriptional level and by Ca2+.
(1) Palmieri, F., et al. 2011 Plant J. 88, 161-181
(2) Fiermonte, G., et al. 2004 J. Biol. Chem. 279, 30722-30730
(3) Palmieri, F., et al. 1995 Methods Enzymol. 260, 349-36
Supplementing the Record: The Life and Career of Judge Edmund L. Palmieri
No full textFor approximately ninety years, lower federal court judges have hired law clerks to process the work of the courts. While the law clerks typically go onto successful careers as attorneys, law professors, government officials, and judges, it is rare that the former apprentices become so famous that their mentors are lost in their oversized shadows. This is the case, however, for former federal district court Judge Edmund L. Palmieri. A highly respected jurist who sat in the Southern District of New York for over three decades, Palmieri has seemingly become the answer to the following trivial pursuit question: What federal judge hired a young Ruth Bader Ginsburg as his law clerk?
While Palmieri should be lauded for offering Ginsburg a position in his chambers, and credited for launching Ginsburg\u27s groundbreaking career, it is unfair to reduce him to an historical footnote in the story of the Notorious RBG. First of all, Ginsburg was neither the first nor the last female law clerk Palmieri hired; during a time when women struggled to find equality of opportunity in the legal profession, almost one-third of the Judge\u27s law clerks were women. And the first three female law clerks hired by Palmieri were Jewish. These progressive hiring practices alone warrant a closer look at the Judge.
Additionally, Palmieri provides a model of what a modern clerkship should be – an invaluable educational opportunity in which a judge serves as role model, teacher, and mentor to newly-minted lawyers. Finally, Palmieri was a loving family man and dedicated public servant who applied his keen intellect to skillfully handle a wide variety of unique cases and novel legal issues which passed through his courtroom. A cultured man who spoke three languages, Palmieri was equally comfortable walking the streets of Brooklyn as he was the sidewalks of the Champs-Élysées. For these reasons, this essay will endeavor to extract Judge Edmund L. Palmieri from the long shadow of his favorite law clerk and return him to his proper place in the history of the federal judiciary
Supplementing the Record: The Life and Career of Judge Edmund L. Palmieri
No full textFor approximately ninety years, lower federal court judges have hired law clerks to process the work of the courts. While the law clerks typically go onto successful careers as attorneys, law professors, government officials, and judges, it is rare that the former apprentices become so famous that their mentors are lost in their oversized shadows. This is the case, however, for former federal district court Judge Edmund L. Palmieri. A highly respected jurist who sat in the Southern District of New York for over three decades, Palmieri has seemingly become the answer to the following trivial pursuit question: What federal judge hired a young Ruth Bader Ginsburg as his law clerk?
While Palmieri should be lauded for offering Ginsburg a position in his chambers, and credited for launching Ginsburg\u27s groundbreaking career, it is unfair to reduce him to an historical footnote in the story of the Notorious RBG. First of all, Ginsburg was neither the first nor the last female law clerk Palmieri hired; during a time when women struggled to find equality of opportunity in the legal profession, almost one-third of the Judge\u27s law clerks were women. And the first three female law clerks hired by Palmieri were Jewish. These progressive hiring practices alone warrant a closer look at the Judge.
Additionally, Palmieri provides a model of what a modern clerkship should be – an invaluable educational opportunity in which a judge serves as role model, teacher, and mentor to newly-minted lawyers. Finally, Palmieri was a loving family man and dedicated public servant who applied his keen intellect to skillfully handle a wide variety of unique cases and novel legal issues which passed through his courtroom. A cultured man who spoke three languages, Palmieri was equally comfortable walking the streets of Brooklyn as he was the sidewalks of the Champs-Élysées. For these reasons, this essay will endeavor to extract Judge Edmund L. Palmieri from the long shadow of his favorite law clerk and return him to his proper place in the history of the federal judiciary
Synergic and Complementary Effects of L-Carnitine and Coenzyme Q on Long-Chain Fatty Acid Metabolism and on Protection against Anthracycline Damage
No full textExogenous L-carnitine and coenzyme Q are used to protect the heart against anthracycline damage and to enhance energy metabolism in the heart and in the muscle. Though their metabolic function is well known and their effects on anthracycline damage have been largely studied, their combined action has not been investigated. Therefore we have used partially CoQ-depleted bovine mitochondria to evaluate the synergic action of CoQ and carnitine on palmitoylCoA oxidation, as an experimental model in which either CoQ or L-carnitine may be the limiting factor in the oxidation of activated fatty acids. The protective effect exerted by the combined use of L-carnitine and CoQ against damage by the anthracycline derivative doxorubicin has been compared to the protection exerted by each compound alone. The effect was evaluated by assessing oxygen consumption and 14C-leucine incorporation in rat heart slices. The results obtained suggest that the administration of an association of L-carnitine and CoQ exerts a stronger protection against anthracycline damage and induces a greater utilization of fatty acids as compared to the effects of each compound alone
- …
