Unreferenced date December 2009 image Mitochondrie.svg thumb 300px Mitochondria structure br 1 Inner membrane br 2 Outer membrane br 3 Crista br 4 Matrix biology Matrix The outer mitochondrial membrane , which encloses the entire organelle, has a protein to phospholipid ratio similar to the eukaryotic plasma membrane about 1 1 by weight . It contains numerous integral protein s called Porin protein porin s , which contain a relatively large internal channel about 2 3 Nanometre nm that is permeable to all molecules of 5000 Atomic mass unit dalton s or less. Larger molecules can only traverse the outer membrane by active transport through mitochondrial membrane transport protein s. The outer membrane also contains enzyme s involved in such diverse activities as the elongation of fatty acid s, oxidation of epinephrine adrenaline , and the Biodegradation degradation of tryptophan . Mitochondrial enzymes See also Bacterial outer membrane MITOL mitochondrial ubiqutin ligase is localized in the mitochondrial outer membrane. DEFAULTSORT Outer Mitochondrial Membrane Category Membrane biology ca Membrana mitocondrial externa zh ... more details
Image Mitochondrie.svg thumb 300px right Mitochondria structure 1 inner membrane, 2 outermembrane, 3 cristae, 4 matrix The mitochondria l inner membrane forms internal compartments known as crista e, which allow greater space for the proteins such as cytochrome s to function properly and efficiently. The electron transport chain is located on the inner membrane of the mitochondria. Within the inner mitochondrialmembrane are also transport protein s that transport in a highly controlled manner metabolites across this membrane. The inner membrane of mitochondria IMM is similar in lipid composition to the membrane of bacteria . This phenomenon can be explained by the endosymbiont hypothesis of the origin of mitochondria as prokaryotes internalized by a eukaryotic host cell. See also Mitochondrial shuttle Translocase of the inner membrane External links http opm.phar.umich.edu localization.php?localization Mitochondrial 20inner 20membrane http www.med.uiuc.edu histo small atlas objects 1340.htm Mitochondrial enzymes Category Membrane biology biochemistry stub ca Membrana mitocondrial interna zh ... more details
Mitochondrialmembrane transport proteins are protein s which exist in the plasma membrane membranes of mitochondria and which serve to transport ref MeshName MitochondrialMembrane 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
SCOP TCDB 3.A.8 OPM family OPM protein PDB The translocase of the outermembrane TOM is a complex of proteins found in the outermitochondrialmembrane of the mitochondria . Its function is to allow ... Functional staging of ADP ATP carrier translocation across the outermitochondrialmembrane journal ... biology cell . The outermembrane of the mitochondrion is impermeable to large molecules greater ... M, Nagase T, et al. title A human homolog of the mitochondrial protein import receptor Mom19 can assemble with the yeast mitochondrial receptor complex journal FEBS Lett. volume 375 issue 3 pages 307 10 year 1996 pmid 7498524 doi 10.1016 0014 5793 95 01229 8 ref The complete mitochondrial protein translocase complex includes at least 19 proteins several chaperones, four proteins of the outermembrane ... space and inner mitochondrialmembrane , contain internal targeting signals, these signals have an indefinable nature and are inconsistent in their pattern. Proteins targeted to the outermembrane also ... mitochondrialmembrane contain a hydrophobic tail domain that anchors the protein to the membrane ... Members of the complex The translocase of the outermembrane TOM forms a complex made of Tom70, TOMM22 ... to the outermembrane by a single transmembrane segment and also plays a role in stabilizing .... title Tom40, the Pore Forming Component of the Protein Conducting Tom Channel in the OuterMembrane ... proteins Category Mitochondria Category Outermembrane proteins es Translocasa de la membrana ...Pfam box Symbol TOM20 plant Name Mitochondrial import receptor subunit TOM20 image PBB Protein TOMM20 ... IPR012621 SMART Prosite SCOP TCDB OPM family OPM protein PDB Pfam box Symbol Tom22 Name Mitochondrial ... ref The TOM works in conjunction with the translocase of the inner membrane TIM to translocate proteins ... membrane translocase Tim and three motor proteins. Protein targeting to the mitochondria There are various mitochondrial import pathways that exist to facilitate the import of precursor proteins ... more details
10.1073 pnas.0402340101 url ref See also Outermitochondrialmembrane References reflist Bacteria InterPro content IPR007485 InterPro content IPR010664 Category Protein families Category Membrane biology ... OPM protein CAZy CDD The bacterial outermembrane is found in Gram negative bacteria . Its composition is distinct from that of the cytoplasmic membrane among other things, the outer leaflet of the outermembrane of many Gram negative bacteria includes a complex lipopolysaccharide whose lipid portion ... van der Ley P, Heckels JE, Virji M, Hoogerhout P, Poolman JT title Topology of outermembrane porins ... of the outermembrane requires that the individual components are transported from the site of synthesis to their final destination outside the inner membrane by crossing both hydrophilic and hydrophobic ... to the outermembrane of Escherichia coli journal J. Bacteriol. volume 190 issue 13 pages ... in very similar outermembrane biogenesis defects. Moreover, the location of at least one of these five ... of lipopolysaccharide LPS from the inner membrane to the outermembrane. ref name pmid18424520 LptE forms a complex with LptD, which is involved in the assembly of LPS in the outer leaflet of the outermembrane and is essential for envelope biogenesis. ref name pmid18424520 ref name pmid16861298 ... of a protein complex that assembles lipopolysaccharide in the outermembrane of Escherichia ... author Bos MP, Tefsen B, Geurtsen J, Tommassen J title Identification of an outermembrane protein required ... ic face of the iner membrane and are separately exported via two independent transport ... ABC transporter MsbA that flips the lipid A core moiety from the inner leaflet to the outer leaflet of the inner membrane. ref name pmid10574995 cite journal author Feldman MF, Marolda CL, Monteiro MA ... more details
Pfam box Symbol OEP Name Outermembrane efflux protein image 1yc9 opm.gif width 200 caption Pfam PF02321 InterPro IPR003423 SMART Prosite SCOP 1ek9 TCDB 1.B.17 OPM family 34 OPM protein 1yc9 PDB Proteins in the outermembrane efflux protein family form trimeric channels that allow export of a variety of substrates in Gram negative bacteria. Each member of this family is composed of two repeats. The trimeric channel is composed of a 12 stranded beta barrel that spans the Bacterial outermembraneoutermembrane , and a long all helical barrel that spans the periplasm . Examples include the Escherichia coli TolC outermembrane protein, which is required for proper expression of outermembrane protein genes the Rhizobium nodulation protein and the Pseudomonas FusA protein, which is involved in resistance to fusaric acid . References Alignment and structure prediction of divergent protein families periplasmic and outermembrane proteins of bacterial efflux pumps. Johnson JM, Church GM J Mol Biol 1999 287 695 715. PMID 10092468 Category Protein domains Category Protein families Category Outermembrane proteins membrane protein stub ... more details
and free fatty acids in the outermembrane ref name PUB00011129 cite journal author Kalk KH, Dijkstra BW, Timmins PA, Snijder HJ title Detergent organisation in crystals of monomeric outermembrane ... in the inner leaflet of the outermembrane. ref InterPro IPR003187 ref . References reflist Category Protein domains Category Outermembrane proteins membrane protein stub ... doi 10.1016 S1047 8477 02 00579 8 ref . The reaction products enhance the permeability of the outermembrane, which allows the semispecific secretion of bacteriocins. One speculative function of OMPLA ... dimerisation. Dimer interactions occur exclusively in the membrane embedded parts of the flat side .... The active site His and Ser residues are located at the exterior of the beta barrel, at the outer leaflet side of the membrane. This location indicates that under normal conditions the substrate and the active ... more details
Pfam box Symbol Porin OmpG Name Outermembrane protein G OmpG image 2iwv.gif width 220 caption Pfam PF09381 InterPro SMART Prosite SCOP 2iwv TCDB 1.B.21 OPM family 181 OPM protein 2iwv PDB Outermembrane protein G OmpG is a porin protein porin , a channel proteins in the Bacterial outermembraneoutermembrane of Gram negative bacteria . Escherichia coli OmpG forms a 14 stranded beta barrel and in contrast to most porins, appears to function as a monomer. ref name pmid16797588 cite journal author Subbarao GV, van den Berg B title Crystal structure of the monomeric porin OmpG journal J. Mol. Biol. volume 360 issue 4 pages 750 9 year 2006 month July pmid 16797588 doi 10.1016 j.jmb.2006.05.045 url ref The central pore of OmpG is wider than other E. coli porins and it is speculated that it may form a non specific channel for the transport of larger oligosaccharide s. ref name pmid16797588 cite journal author Subbarao GV, van den Berg B title Crystal structure of the monomeric porin OmpG journal J. Mol. Biol. volume 360 issue 4 pages 750 9 year 2006 month July pmid 16797588 doi 10.1016 j.jmb.2006.05.045 url ref References reflist Category Protein domains Category Protein families Category Outermembrane proteins membrane protein stub InterPro content ... more details
Pfam box Symbol OpcA Name Outermembrane protein OpcA image 1k24.gif width 200 caption Pfam PF07239 InterPro IPR009876 SMART Prosite SCOP 1k24 TCDB OPM family 27 OPM protein 1k24 PDB PDB3 1k24 A 2 245 Outermembrane adhesin OpcA protein family consists of several Neisseria species specific outermembrane proteins. Neisseria meningitidis causes meningococcal meningitis and septicemia . Opc formerly called 5C is one of the major outermembrane proteins and has been shown to play an important role in meningococcal adhesion and invasion of epithelial and endothelial cells, mediating attachment to host cells by binding proteoglycan cell surface receptor s ref name PUB00013057 cite journal author Zhu P, Klutch MJ, Derrick JP, Prince SM, Tsang RS, Tsai CM title Identification of opcA gene in Neisseria polysaccharea interspecies diversity of Opc protein family journal Gene volume 307 issue pages 31 40 year 2003 pmid 12706886 doi 10.1016 S0378 1119 02 01208 8 ref . OpcA forms a 10 stranded beta barrel with five highly mobile extracellular loops that protrude above the surface of the membrane ref name PUB00028792 cite journal author Derrick JP, Prince SM, Achtman M title Crystal structure of the OpcA integral membrane adhesin from Neisseria meningitidis journal Proc. Natl. Acad. Sci. U.S.A. volume 99 issue 6 pages 3417 21 year 2002 pmid 11891340 doi 10.1073 pnas.062630899 pmc 122538 ref . These extracellular loops combine to form a crevice in the external surface that is lined by positively charged residues, which is predicted to be a binding site for proteoglycan polysaccharides involved in pathogenesis. Conformational changes in the extracellular loops modulate the surface of OpcA, which could affect the proteoglycan binding site ref name PUB00035658 cite journal author Sansom MS, Derrick JP, Bond PJ title Membrane Simulations of OpcA Gating in the Loops? journal Biophys. J. volume ... domains Category Protein families Category Outermembrane proteins InterPro content IPR009876 ... more details
Infobox protein family Symbol Poly export Name Poly export image width caption the structure of wza Pfam PF02563 Pfam clan InterPro IPR003715 SMART PROSITE MEROPS SCOP TCDB 1.B.18 OPM family 188 OPM protein 2j58 CAZy CDD The extracellular polysaccharide colanic acid CA is produced by species of the family Enterobacteriaceae . In Escherichia coli strain K12 the CA cluster comprises 19 genes . The wzx gene encodes a protein with multiple transmembrane segments that may function in export of the CA tandem repeat repeat unit from the cytoplasm into the periplasm in a process analogous to O unit export. The CA Genetic cluster gene cluster s may be involved in the export of polysaccharide from the Cell biology cell . ref name pmid8759852 cite journal author Stevenson G, Andrianopoulos K, Hobbs M, Reeves PR title Organization of the Escherichia coli K 12 gene cluster responsible for production of the extracellular polysaccharide colanic acid journal J. Bacteriol. volume 178 issue 16 pages 4885 93 year 1996 month August pmid 8759852 pmc 178271 doi url ref References reflist InterPro content IPR003715 Category Outer membrane proteins Category Protein families ... more details
Pfam box Symbol OmpW Name image 2f1v.gif width 200 caption Outermembrane protein W Pfam PF03922 InterPro IPR005618 SMART Prosite SCOP TCDB 1.B.39 OPM family 26 OPM protein 2f1v PDB PDB3 2f1v B 23 212 PDB3 2f1t A 23 212 Outermembrane protein W OmpW family is a Protein family family of evolutionarily related proteins from the outer bacterial membrane. This family includes outermembrane protein W OmpW proteins from a variety of bacterial species. This protein may form the receptor for S4 colicin s in Escherichia coli ref name PUB00008474 cite journal author Braun V, Pil sl H, Smajs D title Characterization of colicin S4 and its receptor, OmpW, a minor protein of the Escherichia coli outermembrane journal J. Bacteriol. volume 181 issue 11 pages 3578 3581 year 1999 pmid 10348872 pmc 93827 ref . References reflist Category Protein domains Category Protein families Category Outermembrane proteins membrane protein stub ... more details
Pfam box Symbol Toluene X Name Outermembrane transport proteins OMPP1 FadL TodX image 1t16 opm.gif width 200 caption Structure of the bacterial fatty acid transporter FadL. ref name pmid15178802 cite journal author van den Berg B, Black PN, Clemons WM, Rapoport TA title Crystal structure of the long chain fatty acid transporter FadL journal Science volume 304 issue 5676 pages 1506 9 year 2004 month June pmid 15178802 doi 10.1126 science.1097524 url ref Pfam PF03349 Pfam clan CL0193 InterPro IPR005017 SMART Prosite SCOP TCDB 1.B.9 OPM family 30 OPM protein 1t16 PDB PDB3 1t1l B 28 446 PDB3 1t16 B 28 446 Outermembrane transport proteins OMPP1 FadL TodX Protein family family includes several proteins that are involved in toluene catabolism and degradation of aromatic hydrocarbons. This family also includes protein FadL involved in translocation of long chain fatty acid s across the outermembrane. It is also a receptor for the bacteriophage T2. Notes reflist References Identification of a membrane protein and a truncated LysR type regulator associated with the toluene degradation pathway in Pseudomonas putida F1. Wang Y, Rawlings M, Gibson DT, Labbe D, Bergeron H, Brousseau R, Lau PC Mol Gen Genet 1995 246 570 579. PMID 7535376 Characterization and role of tbuX in utilization of toluene by Ralstonia pickettii PKO1. Kahng HY, Byrne AM, Olsen RH, Kukor JJ J Bacteriol 2000 182 1232 1242. PMID 10671442 p Cymene catabolic pathway in Pseudomonas putida F1 cloning and characterization of DNA encoding conversion of p cymene to p cumate. Eaton RW J Bacteriol 1997 179 3171 3180. PMID 9150211 Category Protein domains Category Protein families Category Outermembrane proteins ... more details
doi 10.1111 j.1365 2958.1991.tb02135.x ref Rck outermembrane protein of the S. typhimurium virulence ... cloacae outermembrane protein OmpX journal J. Bacteriol. volume 173 issue 1 pages 156 160 year 1991 pmid 1987115 pmc 207169 ref a Bacteriophage lambda outermembrane protein, Lom ref name ... is similar to a Yersinia enterocolitica invasion protein and a bacteriophage lambda outermembrane ... The structure of the outermembrane protein OmpX from Escherichia coli reveals possible mechanisms ... sheet topology as the structurally related outermembrane protein A OmpA InterPro IPR000498 , their barrels ... Category Outermembrane proteins membrane protein stub ... of nonpolar residues that attach to the membrane interior. The core of the barrel consists of an extended ... micelle . The OmpX structure shows that the membrane spanning part of the protein is much better conserved ... more details
2 title pH difference across the outermitochondrialmembrane measured with a green fluorescent protein ... transport chain The electron transport chain is located in the cristae of the inner mitochondrialmembrane ... ref Citric acid cycle The citric acid cycle or Krebs cycle or TCA cycle takes place within the mitochondrial ... by oxygen in the matrix to complete the electron transport chain. The protons return to the mitochondrial ... Matrix biology References Reflist Mitochondrial enzymes DEFAULTSORT Mitochondrial Matrix Category Cell ... more details
of ADP ATP carrier translocation across the outermitochondrialmembrane journal J. Biol. Chem. volume ... mitochondrialmembrane inner membranes of mitochondria and other eukaryotic organelles such as the peroxisome ... 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 1okc PDB PDB3 2bmn A 9 104 PDB3 2c3e A 112 206 PDB3 1ymj A 112 206 PDB3 1okc A 112 206 PDB3 1ym6 A 112 206 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 ... 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 ... 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 ... 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 ... dynamics of the mitochondrial ADP ATP carrier revealed by molecular dynamics simulation studies journal ... with respect to the membrane, 3 of them kinked at the level of proline residues. ref name Nury ... pdbe apps quips?story ATPexchange Getting a good rate of exchange the mitochondrial ADP ATP carrier ... 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
or electrical gradients of the membrane process. Membranes can be generally classified into synthetic membrane s and biological membrane s. ref cite book author Mulder, Marcel title Basic principles of membrane technology publisher Kluwer Academic Springer location year 1996 edition 2 isbn 0 7923 4248 8 ref References reflist Category Membrane technology bg cs Membr na de Membran et Membraan es Membrana fr Membrane io Membrano id Membran it Membrana he lv Membr na pl Membrana ... more details
The mitochondrial shuttles are systems used to transport reducing agents across the inner mitochondrialmembrane . NADH cannot cross the membrane, but it can reduce another molecule that can cross the membrane, so that its electrons can reach the electron transport chain . The two main systems in humans are class wikitable Name In, to mitochondrion To electron transport chain ETC Out, to cytosol Glycerol phosphate shuttle glycerol 3 phosphate Coenzyme Q QH sub 2 sub 2 ATP dihydroxyacetone phosphate Malate aspartate shuttle malate Nicotinamide adenine dinucleotide NADH 3 ATP oxaloacetate ref name ufp Silva, Pedro. http www2.ufp.pt pedros bq respi.htm The chemical logic behind... Fermentation and Respiration , Universidade Fernando Pessoa , 2002 01 04. Retrieved on 2009 04 02. ref aspartate In humans, the glycerol phosphate shuttle is primarily found in brown adipose tissue , as the conversion is less efficient, thus generating heat, which is one of the main purposes of brown fat. It is primarily found in babies, though it is present in small amounts in adults around the kidneys and on the back of our necks. ref name ufp The malate aspartate shuttle is found in much of the rest of the body. Notes and references reflist Category Cellular respiration biochem stub ca Llan adora mitocondrial zh ... more details
Membrane penetrating antioxidants have the most important role in improving mitochondrial dysfunction ...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 ... more details
Membrane most commonly means a thin, selective barrier, but it is sometimes used for films that function as separators, like biological membranes. Membrane may also refer to Acoustic membrane Amnion Basement membrane Biological membrane Cell membrane Chorioallantoic membrane Fetal membrane Inner membraneMembraneMembrane keyboard Membrane structure Membrane M Theory Mucous membraneOutermembrane disambiguation Serous membrane Synthetic membrane The Membranes , a punk band Tympanic membrane disambiguation ... more details
marking mtDNA are found near the mitochondrialmembrane. B Whole mount view of cytoplasm after ...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 ... 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
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 mitochondrial protein 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
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
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