Refimprove date January 2008 Infobox Disease Name Mitochondrialtrifunctionalproteindeficiency Image ... 1284 MeshID Mitochondrialtrifunctionalproteindeficiency is an autosomal recessive fatty acid oxidation ... Image autorecessive.svg thumb right Mitochondrialtrifunctionalproteindeficiency has an Recessive ... mitochondrialtrifunctionalproteindeficiency. These genes each provide instructions for making part of an enzyme complex called mitochondrialtrifunctionalprotein. This enzyme complex functions in mitochondria, the energy producing centers within cells. As the name suggests, mitochondrialtrifunctionalprotein contains three enzymes that each perform a different function. This enzyme complex ... in the HADHA or HADHB genes that cause mitochondrialtrifunctionalproteindeficiency disrupt all three ..., heart, and muscles. This abnormal buildup causes the other signs and symptoms of mitochondrialtrifunctionalproteindeficiency. Pathophysiology The mitochondrialtrifunctionalprotein, composed ... and mortality. Epidemiology Mitochondrialtrifunctionalproteindeficiency is a rare disorder its ... long chain fatty acids. Signs and symptoms Signs and symptoms of mitochondrialtrifunctionalproteindeficiency may begin during infancy or later in life. Features that occur during infancy include feeding ... activities. Trifunctionalproteindeficiency is characterized by decreased activity of all 3 enzymes. Clinically, classic trifunctionalproteindeficiency usually results in sudden unexplained infant ... trifunctionalproteindeficiency can be triggered by periods of fasting or by illnesses such as viral ... children with deficiency of LCHAD or complete deficiency of the trifunctionalprotein had been reported ..., breathing difficulties, coma, and sudden death. Signs and symptoms of mitochondrialtrifunctionalproteindeficiency that may begin after infancy include hypotonia, muscle pain, a breakdown of muscle ... deficiency show a protracted progressive course associated with myopathy, recurrent rhabdomyolysis ... more details
Image LCHAD deficiency.jpg right thumb 400px Schematic demonstrating mitochondria l fatty acid beta oxidation and effects of long chain 3 hydroxyacyl coenzyme A dehydrogenase deficiency long chain 3 hydroxyacyl coenzyme A dehydrogenase deficiency, LCHAD deficiency Mitochondrial trifunctional protein is a protein which catalyzes several reactions in beta oxidation . It has two subunits HADHA HADHB The three functions are long chain 3 hydroxy acyl coenzyme A dehydrogenase , 2 enoyl coenzyme A CoA hydratase , and long chain 3 ketoacyl CoA thiolase . ref name urlLong Chain Acyl CoA Dehydrogenase Deficiency eMedicine Pediatrics Genetics and Metabolic Disease cite web url http emedicine.medscape.com article 945857 overview title Long Chain Acyl CoA Dehydrogenase Deficiency eMedicine Pediatrics Genetics and Metabolic Disease format work accessdate 2009 07 11 ref Pathology Disorders are associated with Mitochondrial trifunctional protein deficiency Long chain 3 hydroxyacyl coenzyme A dehydrogenase deficiency LCHAD deficiency References reflist External links MeshName mitochondrial trifunctional protein TP Multienzyme complexes Lipid metabolism enzymes Mitochondrial enzymes Category Proteins protein stub ... more details
. Protein S deficiency is the underlying cause of a small proportion of cases of disseminated ... Protein S Deficiency And Thrombophilia at proteinsdeficiency.com http www.med.uiuc.edu hematology PtProtS.htm Protein S Deficiency University of Illinois Articles cite journal author ten Kate ... PROS1 c.1113T GG frameshift mutation in a family with mixed type I type III protein S deficiency journal ... more details
Mitochondrial membrane transport proteins are protein s which exist in the plasma membrane membranes of mitochondria and which serve to transport ref MeshName Mitochondrial Membrane Transport Proteins ref molecule s and other factors such as ion s into or out of the organelles. Examples Examples of mitochondrial transport protein s include the following The mitochondrial permeability transition mitochondrial permeability transition pore , which opens in response to increased mitochondrial calcium Ca sup 2 sup load and oxidative stress ref name Crompton Crompton M. 1999. http www.biochemj.org bj 341 0233 bj3410233.htm The mitochondrial permeability transition pore and its role in cell death . Biochemical Journal . Volume 341, Pages 233 249. PMID 10393078. Retrieved on January 23, 2007. ref The mitochondrial calcium uniporter which transports calcium in biology calcium from the cytosol of the Cell biology cell into the mitochondrial matrix ref name Crompton ref name Nicholls Nicholls DG. 2005. Mitochondria and calcium signaling. Cell Calcium , Volume 38, Issues 3 4, Pages 311 317. PMID 16087232. Retrieved on January 23, 2007. ref The mitochondrial sodium calcium exchanger , which carries Ca sup 2 sup ions out of the matrix in exchange for sodium Na sup sup ions. These transport proteins serve to maintain the proper electrochemical gradient electrical and chemical gradient s in mitochondria by keeping ions and other factors in the right balance between the inside and outside of mitochondria. See also Mitochondrial carrier Membrane transport protein References references Membrane transport proteins Mitochondrial proteins Category Transport proteins Category mitochondria Category transmembrane proteins membrane protein stub zh ... more details
PBB geneid 64975 39S ribosomal protein L41, mitochondrial is a protein that in humans is encoded by the MRPL41 gene . ref name pmid11543634 cite journal author Kenmochi N, Suzuki T, Uechi T, Magoori M, Kuniba M, Higa S, Watanabe K, Tanaka T title The human mitochondrial ribosomal protein genes mapping of 54 genes to the chromosomes and implications for human disorders journal Genomics volume 77 issue 1 2 pages 65 70 year 2001 month Sep pmid 11543634 pmc doi 10.1006 geno.2001.6622 ref ref name entrez cite web title Entrez Gene MRPL41 mitochondrial ribosomal protein L41 url http www.ncbi.nlm.nih.gov ... cite journal author Yoo YA, Kim MJ, Park JK, et al. title Mitochondrial ribosomal protein L41 suppresses ... author Kim MJ, Yoo YA, Kim HJ, et al. title Mitochondrial ribosomal protein L41 mediates serum starvation ... is automatically maintained by Protein Box Bot. See Template PBB Controls to Stop updates. PBB Summary section title summary text Mammalian mitochondrial ribosomal protein s are encoded by nuclear genes and help in protein synthesis within the mitochondrion . Mitochondrial ribosomes mitoribosomes consist of a small 28S subunit and a large 39S subunit. They have an estimated 75 protein to rRNA composition ... subunit protein that belongs to the YmL27 ribosomal protein family. ref name entrez References reflist ... S, Kitakawa M, Herfurth E, et al. title Mammalian mitochondrial ribosomal proteins. N terminal amino ... mitochondrial ribosome. Analysis of the complement of ribosomal proteins present. journal J. Biol ... journal author Zhang Z, Gerstein M title Identification and characterization of over 100 mitochondrial ribosomal protein pseudogenes in the human genome. journal Genomics volume 81 issue 5 pages 468 ... SR, Jasti M, Malladi S, et al. title BMRP is a Bcl 2 binding protein that induces apoptosis. journal ... gene 9 stub The PBB Controls template provides controls for Protein Box Bot, please see Template PBB Controls for details. PBB Controls update page yes require manual inspection no update protein ... more details
PBB geneid 28977 28S ribosomal protein L42, mitochondrial is a protein that in humans is encoded by the MRPL42 gene . ref name pmid11279123 cite journal author Cavdar Koc E, Burkhart W, Blackburn K, Moseley A, Spremulli LL title The small subunit of the mammalian mitochondrial ribosome. Identification of the full complement of ribosomal proteins present journal J Biol Chem volume 276 issue 22 pages ... Entrez Gene MRPL42 mitochondrial ribosomal protein L42 url http www.ncbi.nlm.nih.gov sites entrez?Db ..., and 15q. ref name entrez cite web title Entrez Gene MRPL42 mitochondrial ribosomal protein L42 url ... maintained by Protein Box Bot. See Template PBB Controls to Stop updates. PBB Summary section title summary text Mammalian mitochondrial ribosomal proteins are encoded by nuclear genes and help in protein synthesis within the mitochondrion. Mitochondrial ribosomes mitoribosomes consist of a small 28S subunit and a large 39S subunit. They have an estimated 75 protein to rRNA composition compared to prokaryotic ..., which prevents easy recognition by sequence homology. This gene encodes a protein identified as belonging ... genes and proteins sequencing and analysis of 500 novel complete protein coding human cDNAs. journal ... cite journal author Kenmochi N, Suzuki T, Uechi T, et al. title The human mitochondrial ribosomal protein genes mapping of 54 genes to the chromosomes and implications for human disorders. journal ... author Koc EC, Burkhart W, Blackburn K, et al. title The large subunit of the mammalian mitochondrial ... author Zhang Z, Gerstein M title Identification and characterization of over 100 mitochondrial ribosomal protein pseudogenes in the human genome. journal Genomics volume 81 issue 5 pages 468 80 year ... U, Lalowski M, et al. title A human proteinprotein interaction network a resource for annotating ... refend gene 12 stub The PBB Controls template provides controls for Protein Box Bot ... no update protein box yes update summary yes update citations yes ... more details
Orphan date July 2011 enzyme Name mitochondrialprotein transporting ATPase EC number 3.6.3.51 CAS number IUBMB EC number 3 6 3 51 GO code 0008566 image width caption In enzymology , a mitochondrialprotein transporting ATPase EC number 3.6.3.51 is an enzyme that catalysis catalyzes the chemical reaction ATP H sub 2 sub O math rightleftharpoons math ADP phosphate Thus, the two substrate biochemistry substrates of this enzyme are adenosine triphosphate ATP and water H sub 2 sub O , whereas its two product chemistry products are adenosine diphosphate ADP and phosphate . This enzyme belongs to the family of hydrolase s, specifically those acting on acid anhydrides to catalyse transmembrane movement of substances. The systematic name of this enzyme class is ATP phosphohydrolase mitochondrialprotein importing . References reflist 1 cite journal author Rassow J year 1997 title Multiple interactions of components mediating preprotein translocation across the inner mitochondrial membrane journal EMBO J. volume 16 pages 2205&ndash 16 pmid 9171336 doi 10.1093 emboj 16.9.2205 last2 Meijer first2 M last3 Maarse first3 AC last4 H nlinger first4 A last5 Dekker first5 PJ last6 Pfanner first6 N last7 Rassow first7 J issue 9 pmc 1169823 cite journal author Brunner M year 1995 title The MIM complex mediates preprotein translocation across the mitochondrial inner membrane and couples it to the mt Hsp70 ATP driving system journal Cell. volume 81 pages 1085&ndash 93 pmid 7600576 doi 10.1016 S0092 8674 05 80013 3 last2 Bauer first2 MF last3 Schneider first3 HC last4 Klaus first4 C last5 Dietmeier first5 K last6 Neupert first6 W last7 Brunner first7 M issue 7 cite journal author Voos W, Martin H, Krimmer T, Pfanner N year 1999 title Mechanisms of protein translocation into mitochondria journal Biochim. Biophys. Acta. volume 1422 pages 235&ndash 54 pmid 10548718 issue 3 Category EC 3.6.3 Category Enzymes of unknown structure hydrolase stub ... more details
PBB geneid 2618 Trifunctional purine biosynthetic protein adenosine 3 is an enzyme that in humans is encoded by the GART gene . ref name pmid2050105 cite journal author Gnirke A, Barnes TS, Patterson D, Schild D, Featherstone T, Olson MV title Cloning and in vivo expression of the human GART gene using yeast artificial chromosomes journal EMBO J. volume 10 issue 7 pages 1629 34 year 1991 month July pmid 2050105 pmc 452831 doi url issn ref This protein is a trifunctional polypeptide. It has phosphoribosylamine glycine ligase phosphoribosylglycinamide formyltransferase EC 6.3.4.13 , AIR synthetase FGAM cyclase phosphoribosylglycinamide synthetase EC 6.3.3.1 , phosphoribosylaminoimidazole synthetase EC 2.1.2.2 activity which is required for de novo purine biosynthesis. References Reflist Further reading refbegin 2 cite journal author Hattori M, Fujiyama A, Taylor TD, et al. title The DNA sequence of human chromosome 21. journal Nature volume 405 issue 6784 pages 311 9 year 2000 pmid 10830953 doi 10.1038 35012518 cite journal author Banerjee D, Nandagopal K title Potential interaction between the GARS AIRS GART Gene and CP2 LBP 1c LSF transcription factor in Down syndrome related Alzheimer disease. journal Cell. Mol. Neurobiol. volume 27 issue 8 pages 1117 26 year 2007 pmid 17902044 doi 10.1007 s10571 007 9217 2 cite journal author Dahms TE, Sainz G, Giroux EL, et al. title The apo and ternary complex structures of a chemotherapeutic target human glycinamide ribonucleotide transformylase. journal Biochemistry volume 44 issue 29 pages 9841 50 year 2005 pmid 16026156 doi 10.1021 bi050307g cite journal author Kimura K, Wakamatsu A, Suzuki Y, et al. title Diversification of transcriptional modulation large scale identification and characterization of putative alternative promoters ... Ewing RM, Chu P, Elisma F, et al. title Large scale mapping of human proteinprotein interactions ... protein involved in de novo purine biosynthesis. journal Mol. Biotechnol. volume 42 issue 3 pages ... more details
Infobox Disease Name Protein C deficiency Image Caption DiseasesDB 10807 ICD10 ICD9 ICD9 289.81 ICDO OMIM 176860 MedlinePlus eMedicineSubj med eMedicineTopic 1923 MeshID D020151 Protein C deficiency is a rare genetic trait that predisposes to thrombosis thrombotic disease . It was first described in 1981 ... C title Deficiency of protein C in congenital thrombotic disease journal J. Clin. Invest. volume 68 ... belongs to a group of genetic disorders known as thrombophilia s. The prevalence of protein C deficiency has been estimated to about 0.2 to 0.5 of the general population. Protein C deficiency is associated ... types of protein C mutations that lead to protein C deficiency ref name pmid16968541 Type I Quantitative defects of protein C low production or short protein half life Type II Qualitative defects ... who develop a venous clot regardless of underlying cause. ref Goldenberg NA, Manco Johnson MJ. Protein C deficiency. Haemophilia. 2008 Nov 14 6 1214 21 ref Studies have demonstrated an increased risk of recurrent venous thromboembolic events in patients with protein C deficiency. Therefore, long ... Johnson MJ. Protein C deficiency. Haemophilia. 2008 Nov 14 6 1214 21 ref Homozygous protein C defect constitutes a potentially life threatening disease, and warrants the use of supplemental protein ... protein C deficiency. ref Pediatr Transplant. 2009 Mar 13 2 251 4. Epub 2008 May 11. Long term survival of a child with homozygous protein C deficiency successfully treated with living donor liver transplantation ... 4 1 15 pmc 1592479 PMC 1592479 ref Pathophysiology The main function of protein C is its anticoagulant ..., factors V VIII and others have been described. The majority of people with protein C deficiency lack only one of the functioning genes, and are therefore heterozygous . Before 1999, only sixteen cases of homozygous protein C deficiency had been described two abnormal copies of the gene, leading to absence of functioning protein C in the bloodstream . This may manifest itself as purpura ... more details
Multiple issues cleanup May 2007 context October 2009 Infobox Disease Name D bifunctional proteindeficiency ... MeshID D Bifunctional proteindeficiency officially called 17 hydroxysteroid dehydrogenase ... proteindeficiency. journal Molecular and Cellular Endocrinology issue 2001 pages 61 70 ref , often ... enzymes their relationship of D bifunctional proteindeficiency and Zellweger syndrome journal Brain ... author S. Buoni, R. Zannolli, H. Waterham, R. Wanders, A. Fois title D Bifunctional ProteinDeficiency ... from D bifunctional proteindeficiency include hypotonia, facial and skull dysmorphism , neonatal ... of D bifunctional proteindeficiency journal Human Molecular Genetics publisher Oxford University Press ... and kidney. D BP deficiency is the most severe peroxisomal disorder ref name ref 5 cite journal ... affected individuals die within the first two years of life. Classification DBP deficiency can be divided ... of D bifunctional protein Defiency and Structure Based Genotype Phenotype Analysis journal The American ... 78 issue 1 ref type I, characterized by a deficiency in both the hydratase and dehydrogenase units of D BP type II, in which only the hydratase unit is non functional type III, with only a deficiency ... were the main cause of these variations of D BP deficiency. Other amino acid changes were seen to alter the dimerization of the protein, leading to improper folding. Many mutations have been found ... notably individuals homozygous for a missense mutation 616S . ref name ref 6 D BP Protein The D bifunctional protein is composed of three enzymatic domains the N terminal short chain alcohol dehydrogenase reductase SDR , central hydratase domain, and the C terminal sterol carrier protein 2 SDR ref name ref 5 . The DBP protein 79kDa also known as multifunctional protein 2 , multifunctional enzyme ... of fatty acids and their derivatives . A non functional D BP protein results in the abnormal accumulation of long chain fatty acids and bile acid intermediates. The D BP protein contains a peroxisomal ... more details
Orphan date July 2011 Infobox protein family Symbol DAP3 Name DAP3 image width caption Pfam PF10236 Pfam clan CL0023 InterPro IPR019368 SMART PROSITE MEROPS SCOP TCDB OPM family OPM protein CAZy CDD In molecular biology, the mitochondrial ribosomal death associated protein 3 family of proteins is a family of conserved sequence conserved proteins which were originally described as death associated protein 3 DAP 3 . The proteins carry a P loop DNA binding motif, and induce apoptosis . ref name pmid9889192 cite journal author Kissil JL, Cohen O, Raveh T, Kimchi A title Structure function analysis of an evolutionary conserved protein, DAP3, which mediates TNF alpha and Fas induced cell death journal EMBO J. volume 18 issue 2 pages 353 62 year 1999 month January pmid 9889192 pmc 1171130 doi 10.1093 emboj 18.2.353 url ref DAP3 has been shown to be a pro apoptotic factor in the mitochondrial matrix ref name pmid11017876 cite journal author Berger T, Brigl M, Herrmann JM, Vielhauer V, Luckow B, Schlondorff D, Kretzler M title The apoptosis mediator mDAP 3 is a novel member of a conserved family of mitochondrial proteins journal J. Cell. Sci. volume 113 issue 20 pages 3603 12 year 2000 month October pmid 11017876 doi url ref and to be crucial for mitochondrion mitochondrial biogenesis and so has also been designated as MRP S29 mitochondrial ribosome ribosomal protein subunit 29 . References reflist InterPro content IPR019368 Category Protein families ... more details
File Catumaxomab mechanism.svg thumb 400px The mechanism of action of a trifunctional antibody, exemplified by catumaxomab A trifunctional antibody is a monoclonal antibody with binding sites for two different ... doi 10.4161 mabs.1.6.10015 ref At an equivalent dose a trifunctional antibody is more potent more ... Jaeger first1 M last2 et al title The trifunctional antibody ertumaxomab destroys tumor cells that express .... These trifunctional antibodies also elicit individual anti tumor immune responses in cancer patients ... of trifunctional anti EpCAM antibody catumaxomab on the development of tumor specific immune ... ascites by the trifunctional antibody catumaxomab results from a pivotal phase II III study journal ... murine hybrid trifunctional bispecific monoclonal antibody for the treatment of cancer journal ... patients with the trifunctional monoclonal antibody catumaxomab anti EpCAM x anti CD3 a phase I study ... I trial of the trifunctional antibody anti HER2 neu x anti CD3 antibody ertumaxomab in metastatic breast ... et al title Immunotherapy of recurrent B cell malignancies after allo SCT with Bi20 FBTA05 , a trifunctional ... effects of the trifunctional bispecific antibody FBTA05 in ex vivo cells of chronic lymphocytic leukaemia ... of the trifunctional bispecific.99702.aspx doi 10.1097 CAD.0b013e328344887f first2 Petra last3 Mueller ... name Ektomab , ref cite journal last1 Ruf first1 P last2 et al title Two new trifunctional antibodies ... of cancer. History Trifunctional antibodies were the first type of bispecific monoclonal antibodies ... indicators for a beneficial clinical outcome. ref cite journal last1 Ott first1 et al title The trifunctional ... The trifunctional antibody catumaxomab for the treatment of malignant ascites due to the epithelial ... types are hybridised, yielding hybrid hybridomas or quadromas , which produce hybrid trifunctional antibody as well as pure mouse and pure rat antibody. The trifunctional antibody is extracted chromatographic ally with protein A . File Quadroma antibodies.svg thumb 400px Possible combinations of light ... more details
to the trifunctional division Odin as the patron of priests and magicians, Thor of warriors ... the trifunctional hypothesis in Greek Epic poetry epic , Lyric poetry lyric and Verse drama and dramatic ... European society Social class Trinity Triple deity DEFAULTSORT Trifunctional Hypothesis Category ... more details
Mitochondria can divide by fission and since they require mitochondrial DNA for their function, fission is coordinated with Mitochondrial DNA Replication DNA replication . Some of the proteins that are involved in mitochondrial fission have been identified and some of them are associated with mitochondrial disease s. ref Cite pmid 21776419 ref Mechanism The DNM1L Drp1 protein is a member of the Dynamin family of large GTPases . Drp1 controls the final part of mitochondrial fission, pinching off the membrane stalk between two forming daughter mitochondria. The MFN2 protein is part of a complex that links the endoplasmic reticulum ER to mitochondria. Points of ER mitochondrial association have been associated with the formation of Drp1 complexes and mitochondrial fission. ref Cite pmid 21885730 ref Several Drp1 binding proteins have been identified. ref Cite pmid 21772324 ref A protein called mitochondrial fission factor Mff binds Drp1 and promotes mitochondrial fission. ref Cite pmid 21149567 ref The FIS1 protein might recruit Drp1 to sites of fission ref Cite pmid 21647385 ref but it might require association with another protein, MIEF1 coded for by the SMCR7L gene , to promote mitochondrial fission. In contrast, MIEF1 when bound to Drp1 might prevent mitochondrial fission and thus shift the balance towards fusion of mitochondria. ref Cite pmid 21701560 ref References reflist Category Mitochondria ... more details
Wiktionary A deficiency is generally a lack of something. It may also refer to A deficient number , in mathematics, a number n for which n 2 n Angular deficiency , in geometry, the difference between a sum of angles and the corresponding sum in a Euclidean plane Deficiency medicine , including various types of malnutrition, as well as genetic diseases caused by deficiencies of endogenously produced proteins. A deficiency in construction , an item, or condition that is considered sub standard, or below minimum expectations Genetic deletion , in genetics, is also called a deficiency A deficiency judgment , in the law of real estate A tax deficiency , an amount owed in taxes over and above what has been submitted in payment disambig ar de Mangel es Deficiencia nl Defici ntie no Mangel ... more details
Mitochondrial biogenesis is the process by which new mitochondria are formed in the cell. Mitochondrial biogenesis is activated by numerous different signals during times of cellular stress or in response to environmental stimuli. The mitochondrion is a key regulator of the metabolic activity of the cell, and is also an important organelle in both production and degradation of free radicals. It is thought that higher mitochodrial copy number or higher mitochondrial mass is protective for the cell. Mitochondria are produced from the transcription and translation of genes both in the nuclear genome and in the Mitochondrial DNA mitochondrial genome . The majority of mitochondrialprotein comes from the nuclear genome, while the mitochondrial genome encodes most parts of the electron transport chain along with mitochondrial rRNA and tRNA . A major adaptation to mitochondrial biogenesis results in more mitochondrial tissues which increases metabolic enzymes for glycolysis, oxidative phosphorylation and ultimately a greater mitochondrial metabolic capacity. Peter D. Wagner Summer 2011 Lecture cn date October 2011 The master regulators of mitochondrial biogenesis appear to be the peroxisome proliferator activated receptor gamma PGC family of transcriptional coactivators, including PPARGC1A PGC 1 , PPARGC1B PGC 1 , and the PGC related coactivator, PRC. PGC 1 , in particular, is thought to be a master regulator. It is known to co activate nuclear respiratory factor 2 GABPA NRF2 GABPA , and together with NRF 2 coactivates nuclear respiratory factor 1 NRF1 . The NRFs, in turn, activate the mitochondrial transcription factor A TFAM tfam , which is directly responsible for transcribing nuclear encoded mitochondrial proteins. This includes both structural mitochondrial proteins as well as those involved in Human mitochondrial genetics Replication.2C repair.2C transcription.2C and translation mtDNA transcription, translation, and repair . See also mitochondria References Unreferenced ... more details
2 title pH difference across the outer mitochondrial membrane measured with a green fluorescent protein ... ref Citric acid cycle The citric acid cycle or Krebs cycle or TCA cycle takes place within the mitochondrial ... transport chain The electron transport chain is located in the cristae of the inner mitochondrial membrane ... by oxygen in the matrix to complete the electron transport chain. The protons return to the mitochondrial matrix through the process of chemiosmosis through the protein ATP synthase . See also Matrix biology References Reflist Mitochondrial enzymes DEFAULTSORT Mitochondrial Matrix Category Cell ... more details
Adenosine diphosphate ADP , Adenosine triphosphate ATP carrier protein ADP ATP translocase 2 oxoglutarate malate carrier protein phosphate carrier protein tricarboxylate transport protein or citrate transport protein Graves disease carrier protein yeast mitochondrial proteins MRS3 and MRS4 yeast mitochondrial FAD carrier protein and many others. Structure All mitochondrial carriers are encoded by nuclear ... families Category Transmembrane proteins membrane protein stub Membrane transport proteins Mitochondrial ...Pfam box Symbol Mito carr Name image 1okc opm.gif width 220 caption Mitochondrial ADP ATP carrier Pfam PF00153 InterPro IPR001993 SMART PROSITE PDOC00189 SCOP 1okc TCDB 2.A.29 OPM family 21 OPM protein ... 206 Mitochondrial carriers are proteins from the solute carrier family which transfers molecules ... of the mitochondrial ADP ATP carrier journal Annu. Rev. Biochem. volume 75 issue pages 713 ... of substrate carrier protein s, which are involved in energy transfer, have been found in the inner mitochondrial membrane inner membranes of mitochondria and other eukaryotic organelles such as the peroxisome ... protein of brown adipose tissue journal Trends Biochem. Sci. volume 15 issue 3 pages ... author Walker JE title The mitochondrial transporter family journal Curr. Opin. Struct. Biol. volume ... journal author Kuan J, Saier Jr MH title Expansion of the mitochondrial carrier family journal Res ... Site directed mutagenesis of the yeast mitochondrial ADP ATP translocator. Six arginines and one lysine ... 10.1006 jmbi.1993.1233 ref ref name PUB00001675 cite journal author Palmieri F title Mitochondrial ... B title PMP47, a peroxisomal homologue of mitochondrial solute carrier proteins journal Trends ... the members of the mitochondrial carrier family that have been identified, it is the ADP ATP carrier ... 10369662 pmc 1171402 doi 10.1093 emboj 18.12.3214 url ref The AAC is an integral membrane protein ... of ADP ATP carrier translocation across the outer mitochondrial membrane journal J. Biol. Chem. volume ... more details
Orphan date December 2010 PBB geneid 94033 Mitochondrial ferritin is a ferroxidase enzyme that in humans is encoded by the FTMT gene . ref name pmid11323407 cite journal author Levi S, Corsi B, Bosisio M, Invernizzi R, Volz A, Sanford D, Arosio P, Drysdale J title A human mitochondrial ferritin encoded by an intronless gene journal J. Biol. Chem. volume 276 issue 27 pages 24437 40 year 2001 month July pmid 11323407 doi 10.1074 jbc.C100141200 url issn ref It is classified as a metal binding protein which is located within the mitochondrion mitochondria . After the protein is taken up by the mitochondria it can be processed into a mature protein and assemble functional ferritin shells. Structure Its structure was determined at 1.70 through the use of X ray diffraction and contains 182 residues. It is 67 helical. The Ramachandran plot shows that the structure of mitochondrial ferritin is mainly alpha helical with a low prevalence of beta sheets. References reflist 2 Further reading refbegin ... and biochemical properties of the human mitochondrial ferritin and its mutant Ser144Ala. journal ... in HeLa cells are rescued by the expression of human mitochondrial ferritin. journal Biochim. Biophys ... 59Fe in cells when the protein pores are unfolded by mutation. journal J. Biol. Chem. volume 283 ... author Cazzola M, Invernizzi R, Bergamaschi G, et al. title Mitochondrial ferritin expression in erythroid ... R, et al. title Mitochondrial ferritin a new player in iron metabolism. journal Blood Cells Mol. Dis ... P, et al. title Mitochondrial ferritin limits oxidative damage regulating mitochondrial iron ... EL, Ray PD, Tsuji Y title Role and regulation of ferritin H in rotenone mediated mitochondrial ... P, Levi S, et al. title Unique iron binding and oxidation properties of human mitochondrial ... SM, et al. title Mitochondrial ferritin in the substantia nigra in restless legs syndrome. journal ... 10.1097 NEN.0b013e3181bdc44f refend DEFAULTSORT Mitochondrial Ferritin Category EC 1.16.3 Category ... more details
Image Mitochondrial DNA en.svg thumb 300px right Human mitochondrial DNA. File Electron microscopy reveals mitochondrial DNA in discrete foci.jpg thumb Electron microscopy reveals mitochondrial DNA in discrete ... marking mtDNA are found near the mitochondrial membrane. B Whole mount view of cytoplasm after ... H, Cook PR title The functional organization of mitochondrial genomes in human cells journal BMC ... www.biomedcentral.com 1741 7007 2 9 ref Mitochondrial DNA mtDNA or mDNA ref cite web last Sykes first B title Mitochondrial DNA and human history url http genome.wellcome.ac.uk doc WTD020876.html work ... . Mitochondrial DNA can be regarded as the smallest chromosome , and was the first significant part ... has become important in anthropology and field biology. Origin Nuclear and mitochondrial DNA are thought ... target molecules by exponential polymerase chain reaction, copy number of mitochondrial DNA in rat ... origin, having since been transferred to the eukaryotic nucleus during evolution . Mitochondrial ... injecting a sperm into an oocyte , may interfere with this. The fact that mitochondrial DNA ... region s HVR1 or HVR2 of the mitochondrial DNA, as with a genealogical DNA test . HVR1 consists ... issn 0036 8075 pmid 9180076 issue 5319 ref The concept of the Mitochondrial Eve is based ... limited biparental inheritance of Mytilus mitochondrial DNA journal Science volume 251 pages 1488 1490 ... mitochondrial DNA by PCR selective amplification method, journal Genet. Res. volume 59 issue ... Meusel MS, Moritz RF title Transfer of paternal mitochondrial DNA during fertilization of honeybee ... of male mitochondrial inheritance in some mammals as well. Specifically, documented occurrences ... inheritance of mitochondrial DNA in mice journal Nature volume 352 issue 6332 pages 255 7 year 1991 ... X, Li N, Guo W, et al. title Further evidence for paternal inheritance of mitochondrial DNA in the sheep ... J, et al. title Composition of parental mitochondrial DNA in cloned bovine embryos journal FEBS ... more details
Unreferenced section date December 2011 Infobox Disease Name Mitochondrial disease Image Ragged red fibres gtc very high mag.jpg Caption Micrograph showing ragged red fibres, a finding seen in mitochondrial ... Mitochondrial diseases are a group of disorders caused by dysfunctional mitochondria , the organelle ... ATP that powers most cell functions. Mitochondrial diseases are often caused by genetics or mutations to the mitochondrial DNA that affect mitochondria function. Mitochondrial diseases take on unique ... symptoms are often called a mitochondrial myopathy . Classification In addition to the mitochondrial ... age can be due to mitochondrial disease Diabetes mellitus and deafness can also be found together for other ... exercise intolerance MELAS Mitochondrial myopathy, encephalomyopathy, lactic acidosis, stroke like symptoms MELAS mtDNA depletion mitochondrial neurogastrointestinal encephalomyopathy MNGIE Nota bene Conditions such as Friedreich s ataxia can affect the mitochondria , but are not associated with mitochondrial ..., and dementia. Characteristics The effects of mitochondrial disease can be quite varied. Since the distribution of the defective mitochondrial DNA may vary from organ to organ within the body, and each ... severely, leading to multi system diseases. Mitochondrial diseases as a rule are worse when the defective ... author Finsterer J title Hematological manifestations of primary mitochondrial disorders journal ... because these cells use more energy than most other cells in the body. Although mitochondrial diseases ... consequences. Causes Mitochondrial disorders may be caused by mutations , acquired or inherited, in mitochondrial DNA mtDNA or in nuclear genes that code for mitochondrial components. They may also be the result of acquired mitochondrial dysfunction due to adverse effects of drugs , infections ... the mother. Mitochondrial DNA, however, is strictly inherited from the mother and each mitochondrial organelle typically contains multiple mtDNA copies see Heteroplasmy . During cell division the mitochondrial ... more details
Infobox Disease Name Mitochondrial encephalomyopathy Image Caption DiseasesDB ICD10 ICD9 ICD9 277.87 ICDO OMIM MedlinePlus eMedicineSubj eMedicineTopic MeshID D017237 A Mitochondrial encephalomyopathy is a form of encephalomyopathy that is associated with a mitochondrial disease . Examples include Mitochondrial encephalomyopathy, lactic acidosis, and stroke like episodes MELAS and MERRF syndrome MERRF . These conditions can sometimes present together. ref name pmid14967777 cite journal author Melone MA, Tessa A, Petrini S, et al title Revelation of a new mitochondrial DNA mutation G12147A in a MELAS MERFF phenotype journal Arch. Neurol. volume 61 issue 2 pages 269 72 year 2004 month February pmid 14967777 doi 10.1001 archneur.61.2.269 url http archneur.ama assn.org cgi pmidlookup?view long&pmid 14967777 ref ref name pmid15767514 cite journal author Naini AB, Lu J, Kaufmann P, et al title Novel mitochondrial DNA ND5 mutation in a patient with clinical features of MELAS and MERRF journal Arch. Neurol. volume 62 issue 3 pages 473 6 year 2005 month March pmid 15767514 doi 10.1001 archneur.62.3.473 url http archneur.ama assn.org cgi pmidlookup?view long&pmid 15767514 ref Kearns Sayre syndrome KSS is sometimes included in this category, ref name pmid8350109 cite journal author Crimmins D, Morris JG, Walker GL, et al title Mitochondrial encephalomyopathy variable clinical expression within a single kindred journal J. Neurol. Neurosurg. Psychiatr. volume 56 issue 8 pages 900 5 year 1993 month August pmid 8350109 pmc 1015147 doi 10.1136 jnnp.56.8.900 url http jnnp.bmj.com cgi pmidlookup?view long&pmid 8350109 ref but it is not included in this category in Medical Subject Headings MeSH . References reflist 2 Mitochondrial diseases Category Neurotrauma disease stub zh ... more details
Mitochondrial toxicity is a condition in which the mitochondrion mitochondria of a body s cells become damaged or decline significantly in number it occurs as a side effect of certain antiretroviral drug s used to treat human immunodeficiency virus, or HIV . Causes While the exact causes of mitochondrial toxicity are not known, research has shown that a certain group of anti viral drugs used to treat HIV, specifically nucleoside analog reverse transcriptase inhibitor s NRTIs , interfere with an enzyme needed in the production of mitochondria. ref http www.thebody.com pinf mitochondrial.html ref Symptoms The disruption of cell function that accompanies the condition can cause both mild and severe problems in people suffering from mitochondrial toxicity. The most commonly observed symptom is muscle weakness, or myopathy . Others include peripheral neuropathy numbness in the fingers and toes and pancreatitis inflammation of the pancreas , with the most severe being lactic acidosis , in which a build up of lactic acid in the tissues of the body leads to loss of energy, organ failure, and eventually death. ref http www.projinf.org fs mito b.html ref References references External links Mitochondrial toxicity at http www.hivnet.org OverHivEnAids Alternatief BrochureBijwerkingenEngels Mitochondrial 20toxicity.htm hiv.org Category Toxicology medical stub med toxic stub sl Mitohondrijska toksi nost ... more details
Infobox album See Wikipedia WikiProject Albums Name Mitochondrial Blues Type Album Artist Noah23 Cover Noah23 mitochondrial blues.jpg Released July 27, 2004 Recorded 2003 2004 Genre Hip hop music Hip hop Length 56 26 Label Legendary Entertainment br Noah23 Plague Language Producer Blue Sky Black Death Orphan , Sseleman, Noah23 , Andrew H, DMS, Krinjah, King Champion, Naught, Dustmite Last album Ancient Israelites Older Than Anorthosite br 2004 This album Mitochondrial Blues br 2004 Next album Sigma Octantis album Sigma Octantis br 2004 Mitochondrial Blues is a 2004 album by hip hop artist Noah23 . This album was released simultaneously with Sigma Octantis album Sigma Octantis , as both albums are the product of the same recording sessions as Noah s higher profile 2004 album Jupiter Sajitarius . Track listing Silicate Magma Chamber Crunch Search and Destroy Harvest Remix Orange Dolphin Pure Vibes ft. Fake Four Inc Ceschi & Troubadour Twist of Fate ft. Baracuda rapper Baracuda Homewood ft. Feelix Enterworld ft. B One & Vex Bliss TS4 Freestyle Mechanical Bull ft. Baracuda Yed Posterior ft. Erosadis, Hangnail & Homesick Dustmite Data Hooks ft. Baracuda Drowning in You ft. AnuCongo Trilateral Damage ft. Orko the Sykotik Alien & StapleMouth Production credits Tracks 1, 5, 7, 14 & 16 produced by Blue Sky Black Death Orphan Tracks 6, 9 & 15 produced by Sseleman Track 2 produced by Noah23 as Warhol Track 3 produced by Andrew H Track 4 produced by DMS Track 8 produced by Krinjah Track 11 produced by King Champion Track 12 produced by Naught Track 13 produced by Dustmite Category 2004 albums Category Noah23 albums 2000s hiphop album stub ... more details
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