Mitochondria are double membraned organelles responsible for the majority of ATP production in eukaryotic cells. The mitochondrial inner membrane is folded into cristae structures and is the site of the electron transport chain which terminates in ATP generation. ATP is produced by ATP synthase, a protein complex that has also been shown to have a role in the maintenance of cristae folding. This activity is dependent on Tim11p, a subunit required for the dimerization of ATP synthase super-complexes. Additional proteins located within the inner membrane that are important to mitochondrial morphology include Pcp1p, a serine protease, and its substrate, Mgm1p. Mgm1p is required for mitochondrial fusion and cells deleted for MGM1 do not contain detectable Tim11p. Using biochemical assays and transmission electron microscopy, this study characterized pcp1 mutants in order to analyze the link between Pcp1p functionality, ATP synthase super-complex assembly, and mitochondrial morphology.

No description available.

Assignment (MMed)--University of Stellenbosch, 2007. / ENGLISH ABSTRACT: Lipoprotein X (LpX) is an abnormal cholesterol-containing particle that may be present in the serum of subjects with cholestasis, lecithin:cholesterol acyltransferase (LCAT) deficiency and parenteral nutrition. The biochemistry, metabolism, clinical significance and laboratory analysis of LpX is discussed in this study. This laboratory-based project investigated icteric samples received at the Chemical Pathology laboratory, Tygerberg Hospital, for serum predictors of LpX and the use of a modified non-denaturing polyacrylamide gradient gel electrophoresis system in the detection of LpX. The study showed that the non-denaturing polyacrylamide gradient gel electrophoresis system (2-8%) is a useful test in demonstrating LpX in icteric plasma and has potential for a screening test in LCAT deficiency. Serum concentration of conjugated bilirubin, alkaline phosphatase, gamma glutamyltransferase, free cholesterol, phospholipid, free cholesterol: total cholesterol ratio and conjugated bilirubin: total bilirubin ratio are all good predictors of LpX. The ratio of free cholesterol to total cholesterol (FC/TC > 0.6) was the best predictor of LpX. In the setting of obstructive liver disease LpX is seen in 66% of patients if total cholesterol is > 7.5 mmol/L. / AFRIKAANSE OPSOMMING: Lipoproteien X (LpX) is ‘n abnormale cholesterol-bevattende partikel wat teenwoordig mag wees in die serum van persone met cholestase, lesitien:cholesterol asieltransferase (LCAT) gebrek en parenterale voeding. Die biochemie, metabolisme, kliniese belang en laboratorium analise van LpX word bespreek in hierdie werkstuk. Hierdie laboratorium-gebaseerde projek het geelsugtige monsters ondersoek wat ontvang is by die Chemiese Patologie laboratorium, Tygerberg Hospitaal, vir serum voorspellers van LpX en die gebruik van ‘n gemodifiseerde nie-denaturerende polie-akrielamied gradiënt gel elektroforese sisteem in die demonstrasie van LpX. Die bevindinge was dat die nie-denaturerende polie-akrielamied gradient gel elektroforese sisteem (2-8%) is ‘n nuttige toets om LpX te demonstreer in geelsugtige plasma en het potensiaal as ‘n siftingstoets in LCAT gebrek. Serum konsentrasie van gekonjugeerde bilirubien, alkaliese fosfatase, gamma glutamieltransferase, vry cholesterol, fosfolipied, vry cholesterol:totale cholesterol verhouding en gekonjugeerde bilirubien:totale bilirubien verhouding is alles goeie voorspellers van LpX. Die verhouding van vry cholesterol tot totale cholesterol (VC/TC > 0.6) was die beste voorspeller van LpX. In gevalle van obstruktiewe lewersiekte word LpX gesien in 66% van pasiente as die totale cholesterol meer as 7.5 mmol/l is.

No description available.

Thesis (Ph. D.)--University of Washington, 2006. / Vita. Includes bibliographical references (leaves 106-113).

Haptoglobin is an acute phase protein with important biological role because of its capacity to bind to haemoglobin. Haptoglobin exists in three major genetic polymorphism types: Hp1-1, Hp2-1 and Hp2-2, the distribution of which has been associated with abdominal aortic aneurysm (AAA), an asymptomatic aortic disease common among men older than 65 years.    Five different electrophoretic methods were tested according to their ability to separate the haptoglobin phenotypes. The detection was based on a produced hemolysate of blood in which haemoglobin binds to haptoglobin thereby forming a complex that can be detected by specific haemoglobin staining using TMB-dihydrochloride and hydro peroxide as substrate resulting in an azure-green color of the bands. Samples from 15 patients who had suffered surgery for not broken AAA, that is more than5.0 cmaortic diameter, and 15 samples from matched controls were analyzed.    Among the five tested electrophoretic methods best migration and separation was seen on the pre-cast agarosgel Hydragel HR on the instrument Hydrasys. The other four methods gave less successful results. This pilot investigation showed the following distribution of the phenotypes of haptoglobin among AAA patients; 7 % Hp1-1, 40 % Hp2-1 and 53 % Hp2-2 and for the controls; 13 % Hp1-1, 33 % Hp2-1 and 53 % Hp2-2.    In conclusion, the used techniques has to be further optimized and more patients have to be included in the study before it can be ascertained if the phenotypes of Haptoglobin play any role in the progress of the AAA disease.

Thesis (M.S.)--Rochester Institute of Technology, 1989. / Includes bibliographical references (leaves 81-83).

Thesis (Ph. D.)--University of Washington, 1999. / Vita. Includes bibliographical references (leaves 82-88).

Made available in DSpace on 2015-04-09T12:28:12Z (GMT). No. of bitstreams: 0 Previous issue date: 2013-02-08Bitstream added on 2015-04-09T12:48:01Z : No. of bitstreams: 1 000814587.pdf: 233099 bytes, checksum: 86c73283cfc6d5c2954be91ce17d3f29 (MD5) / Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) / Endotoxins are often found in high concentration in the peritoneal fluid from horses with acute gastrointestinal disease and blood of septicemic foals. The conditions mentioned earlier can lead to inflammatory processes and cause changes in the concentrations of acute phase proteins. The objectives of this study were to determine by means of polyacrylamide gel electrophoresis (SDS-PAGE), the concentrations of acute phase proteins (transferrin, ceruloplasmin, albumin, haptoglobin and acid glycoprotein) in serum and peritoneal fluid of horses submitted to experimental endotoxemia and evaluate the possible effects of the administration of lidocaine on the production of these proteins after intraperitoneal injection of endotoxin in these animals. To this end, 12 animals were distributed in two groups, control which was treated with 0.9% NaCl solution and the experimental which was treated with continuous infusion for 6 hours of 2% lidocaine solution. It is concluded that in this experimental model the intraperitoneal administration of endotoxin caused an increase in the concentrations of peritoneal transferrin, ceruloplasmin and haptoglobin in the control group, of albumin in both groups, and the acid glycoprotein in the group treated with infusion of lidocaine. However, the absence of changes in the concentrations of serum ceruloplasmin, serum albumin, haptoglobin and acidic glycoprotein indicate that the amount of the circulatory endotoxin was not enough to alter serum concentrations of these proteins. The continuous infusion of lidocaine interfered in peritoneal acute phase proteins values and had an anti-endotoxemic effect / FAPESP: 2009/15683-0

Resumo:As endotoxinas são frequentemente detectadas em alta concentração no líquido peritoneal de equinos com doença gastrintestinal aguda e no sangue de potros septicêmicos. As condições citadas anteriormente podem levar a processos inflamatórios e provocar alterações nas concentrações de proteínas de fase aguda. Os objetivos deste estudo foram determinar, por meio de eletroforese em gel de poliacrilamida (SDS-PAGE), as concentrações de proteínas de fase aguda (transferrina, ceruloplasmina, albumina, haptoglobina e glicoproteína ácida) no soro e no líquido peritoneal de equinos submetidos à endotoxemia experimetal, e avaliar os possíveis efeitos da administração da lidocaína sobre a produção destas proteínas depois da injeção intraperitoneal de endotoxina nestes animais. Para tanto, foram utilizados 12 animais distribuídos em dois grupos, o controle, tratado com solução de NaCl a 0,9%, e o experimental, tratado com infusão contínua de solução de lidocaína a 2% por 6 horas. Conclui-se que neste modelo experimental a administração intraperitoneal de endotoxina causou o aumento nas concentrações peritoneais de transferrina, ceruloplasmina e haptoglobina no grupo controle, da albumina em ambos os grupos, e da glicoproteína ácida no grupo tratado com infusão de lidocaína. Porém a ausência de alterações nas concentrações séricas de ceruloplasmina, albumina, haptoglobina e glicoproteína ácida indicam que a quantidade de endotoxina que chegou à corrente circulatória não foi suficiente para alterar as concentrações séricas destas proteínas. A infusão contínua de lidocaína interferiu nos valores peritoneais das proteínas mensuradas, tendo efeito anti-endotoxêmico / Abstract:Endotoxins are often found in high concentration in the peritoneal fluid from horses with acute gastrointestinal disease and blood of septicemic foals. The conditions mentioned earlier can lead to inflammatory processes and cause changes in the concentrations of acute phase proteins. The objectives of this study were to determine by means of polyacrylamide gel electrophoresis (SDS-PAGE), the concentrations of acute phase proteins (transferrin, ceruloplasmin, albumin, haptoglobin and acid glycoprotein) in serum and peritoneal fluid of horses submitted to experimental endotoxemia and evaluate the possible effects of the administration of lidocaine on the production of these proteins after intraperitoneal injection of endotoxin in these animals. To this end, 12 animals were distributed in two groups, control which was treated with 0.9% NaCl solution and the experimental which was treated with continuous infusion for 6 hours of 2% lidocaine solution. It is concluded that in this experimental model the intraperitoneal administration of endotoxin caused an increase in the concentrations of peritoneal transferrin, ceruloplasmin and haptoglobin in the control group, of albumin in both groups, and the acid glycoprotein in the group treated with infusion of lidocaine. However, the absence of changes in the concentrations of serum ceruloplasmin, serum albumin, haptoglobin and acidic glycoprotein indicate that the amount of the circulatory endotoxin was not enough to alter serum concentrations of these proteins. The continuous infusion of lidocaine interfered in peritoneal acute phase proteins values and had an anti-endotoxemic effect / Orientador: Francisco Leydson Formiga Feitosa / Banca: Marcos Pinheiro Franque / Banca: Taciana Rabelo Ramalho Ramos / Banca: Paulo Sérgio Patto dos Santos / Banca: Juliana Regina Peiró / Doutor