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
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
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 . There is some evidence for mitochondrial dysfunction as the molecular basis of bipolar disorder. In addition, classical mitochondrial diseases occur in a subset of individuals with autism and are usually caused by genetic anomalies or mitochondrial respiratory pathway deficits. Classification In addition to the mitochondrial myopathies, other examples include Diabetes mellitus and deafness DAD this combination at an early age can be due to mitochondrial ... 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 proteins .... 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 mutation is modulated ... to multi system diseases. Mitochondrial diseases as a rule are worse when the defective mitochondria ... J title Hematological manifestations of primary mitochondrial disorders journal Acta Haematol ... use more energy than most other cells in the body. Although mitochondrial diseases vary greatly in presentation .... 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 ... more details
Symptomatic overlap with other mitochondrial myopathies References reflist Diseases of myoneural junction and muscle Mitochondrial diseases Category Myoneural junction and neuromuscular diseases Category Mitochondrial diseases zh ... 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
Expert subject Biology date November 2009 No footnotes date May 2011 Mitochondrial decay is one major cause of aging , leading to the subsequent death in humans. The mitochondria are the power producing organelles found in every cell of every organ. Their primary function is to generate Adenosine triphosphate ATP through various energy cycles that involve nutrients and vitamins. ATP is needed for every movement, thought and action we make. Yet very little ATP can be stored in the body. Under normal conditions the mitochondria generate free radicals during the ATP production process and as a result subject themselves to a lot of free radical damage. As we age, the mitochondria become less efficient, fewer in number and larger. Accordingly, ATP production declines, and this may eventually lead to cell death. As organs cannot borrow energy from one another, the efficiency of each organ s mitochondria is essential to its repair processes and functions. If an organ s mitochondria fail, then so does that organ. Enhancement and protection of mitochondrial function and structure are therefore instrumental in preventing and slowing aging. Mitochondrial decay is a progressive disruption of mitochondrial structural integrity and functional ability with advancing age. It can cause impairment in cellular energy production and hence a decline in normal function, particularly in organs with high energy consumption such as the brain and the heart. Mitochondrial decay can also lead to an increase in free radical production and thus render a cell more prone to commit suicide programmed cell death or apoptosis . Some of the age related diseases, including cardiovascular and neurodegenerative disorders, have been found to involve mitochondrial dysfunction. The maintenance of a youthful mitochondrial functional ability and antioxidant capacity has become a trend in preventive health. Reference RK Lab http www.rk lab.com Category DNA Category Aging ... more details
pH difference across the outer mitochondrial membrane measured with a green fluorescent protein ... or TCA cycle takes place within the mitochondrial matrix. Prior to the citric acid cycle, pyruvic ... of the inner mitochondrial membrane. The NADH and FADH sub 2 sub produced by the citric acid ... 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 anatomy ca Matriu mitocondrial cs Mitochondri ln matrix ... more details
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 across the membranes of the mitochondria . ref name Nury 2006 cite journal author Nury H, Dahout Gonzalez C, Tr z guet V, Lauquin GJ, Brandolin G, Pebay Peyroula E title Relations between structure and function of the mitochondrial ADP ATP carrier journal Annu. Rev. Biochem. volume 75 issue pages 713 ... mitochondrial membrane inner membranes of mitochondria and other eukaryotic organelles such as the peroxisome ... 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 ... of ADP ATP carrier translocation across the outer mitochondrial membrane journal J. Biol. Chem. volume ... dynamics of the mitochondrial ADP ATP carrier revealed by molecular dynamics simulation studies journal ... pdbe apps quips?story ATPexchange Getting a good rate of exchange the mitochondrial ADP ATP carrier ... families Category Transmembrane proteins membrane protein stub Membrane transport proteins Mitochondrial ... 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
Selection An Improved Human Mitochondrial Molecular Clock. and its http download.cell.com AJHG ... Human mitochondrial DNA haplogroup Mitochondrial macro haplogroups Haplogroup L0 mtDNA L0 ... , Mitochondrial Eve refers to the matrilineal MRCA most recent common ancestor . In other words, she ... all mitochondrial DNA mtDNA is generally passed from mother to offspring without recombination biology recombination , all mitochondrial DNA mtDNA in every living person is directly descended from hers by definition. Mitochondrial Eve is the female counterpart of Y chromosomal Adam , the patrilineal ... or more males in their descendancy line . But none of the female contemporaries of the Mitochondrial Eve has descendants living now in an unbroken female line. Mitochondrial Eve is estimated to have ... modern humans were developing as a population distinct from other human sub species. Mitochondrial ... C title Evaluating the mitochondrial timescale of human evolution journal Trends Ecol. Evol. Amst. volume ... . As a result, the latter hypothesis is now the dominant one. Female and mitochondrial ancestry further Genetic genealogy matrilineal Mitochondrial DNA Human mitochondrial molecular clock File ... back to a single female, such as Mitochondrial Eve File Mitochondrial eve tree.gif thumb right 250px Simplified Human mitochondrial phylogeny Without a DNA sample, it is not possible to reconstruct ... DNA, however, parts of ancestral genomes are estimated by scientists. Mitochondrial DNA mtDNA ... title Maternal inheritance of human mitochondrial DNA journal PNAS volume 77 issue 11 pages 6715 ... give a mitochondrial DNA signature or haplotype e.g. the Cambridge Reference Sequence CRS is a haplotype ... mutation . Scientists sort mitochondrial DNA results into more or less related groups, with more or less ... are in biological terms clade s, and the common ancestors such as Mitochondrial Eve sit at branching ... . The mitochondrial clade which Mitochondrial Eve defines is the species species Homo sapiens Homo ... 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 is the DNA located in organelles called ... organisms is found in the cell nucleus . Mitochondrial DNA can be regarded as the smallest ... John JC, Facucho Oliveira J, Jiang Y, Kelly R, Salah R title Mitochondrial DNA transmission, replication ... 20231166 doi 10.1093 humupd dmq002 url ref Origin Nuclear and mitochondrial DNA are thought to be of separate ... by exponential polymerase chain reaction, copy number of mitochondrial DNA in rat tissues journal ... since been transferred to the eukaryotic nucleus during evolution . Mitochondrial inheritance In most ... a sperm into an oocyte , may interfere with this. The fact that mitochondrial DNA is maternally ... or HVR2 of the mitochondrial DNA, as with a genealogical DNA test . HVR1 consists of about 440 base ... issn 0036 8075 pmid 9180076 issue 5319 ref The concept of the Mitochondrial Eve is based on the same ... inheritance of Mytilus mitochondrial DNA journal Science volume 251 pages 1488 1490 pmid 1672472 ... R, Matsuura ET, Chigusa SI title Further observation of paternal transmission of Drosophila mitochondrial ..., Moritz RF title Transfer of paternal mitochondrial DNA during fertilization of honeybee Apis mellifera ... editor1 last Crusio editor1 first Wim ref Evidence supports rare instances of male mitochondrial ... of mitochondrial DNA in mice journal Nature volume 352 issue 6332 pages 255 7 year 1991 pmid 1857422 ... W, et al. title Further evidence for paternal inheritance of mitochondrial DNA in the sheep Ovis aries ... al. title Composition of parental mitochondrial DNA in cloned bovine embryos journal FEBS Lett. 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 2 cite journal author Langlois d Estaintot B, Santambrogio P, Granier T, et al. title Crystal structure 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 ... 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
The mitochondrial shuttles are systems used to transport reducing agents across the inner mitochondrial membrane . 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
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 56947 Mitochondrial fission factor Mff is a protein that in humans is encoded by the MFF gene . ref name entrez cite web title Entrez Gene mitochondrial fission factor url http www.ncbi.nlm.nih.gov sites entrez?Db gene&Cmd ShowDetailView&TermToSearch 56947 accessdate ref Mff is an outer mitochondrial membrane protein that binds to the DNM1L Drp1 GTPases GTPase that controls mitochondrial fission . The Mff Drp1 complex promotes mitochondrial fission. ref Cite pmid 21149567 ref Mff also regulates peroxisome morphology. ref Cite pmid 18353969 ref References reflist Further reading refbegin cite journal author Igci YZ, Arslan A, Akarsu E, et al. title Differential expression of a set of genes in follicular and classic variants of papillary thyroid carcinoma. journal Endocr. Pathol. volume 22 issue 2 pages 86 96 year 2011 pmid 21509594 doi 10.1007 s12022 011 9157 8 refend gene 2 stub Category Mitochondria ... more details
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
Orphan date September 2010 Infobox Journal title Mitochondrial DNA editor Rob DeSalle discipline CellBiology Genetics Evolution language English abbreviation MDN publisher Informa Healthcare Country UK Frequency 6 issues per year History First published 1990 openaccess no website http www.informahealthcare.com mdn impact 1.273 impact year 2010 link1 link1 name link2 link2 name RSS atom JSTOR OCLC LCCN CODEN ISSN 1940 1736 eISSN 1940 1744 Mitochondrial DNA is an academic journal that publishes Review journal review articles on the current and developing technologies around mitochondrial DNA research and discovery. It is published by Informa plc Informa Healthcare . Core Areas Coverage includes Discovery and description of mitochondrial genome sequences Structure and function of mtDNA genes and genomic features Molecular evolution, phylogenetics, population genetics Model and non model organisms ... and Scope Previously published under the title DNA Sequence Vols 1 19.3 , Mitochondrial DNA accepts original high quality reports based on mapping, sequencing and analysis of mitochondrial DNA and RNA. Descriptive papers on DNA sequences from mitochondrial genomes, and also analytical papers in the areas of population genetics, medical genetics, phylogenetics and human evolution that use mitochondrial ... address the use of mitochondrial DNA sequences, as well as papers that discuss the utility of mitochondrial ... desalle.amnh.org Rob DeSalle American Museum of Natural History is the Editor in Chief of Mitochondrial ... koloko.net Sergios Orestis Kolokotronis Barnard College, Columbia University . Special Issues Mitochondrial ... http informahealthcare.com toc mdn 22 S1 ref Publication Format Mitochondrial DNA publishes WebFirst ... to the online version is included in all subscriptions. Abstracting & Indexing Mitochondrial ... Scopus Zoological Record . References reflist 2 External links http www.informahealthcare.com mdn Mitochondrial DNA homepage of Mitochondrial DNA Category Publications established in 1980 Category Genetics ... more details
refimprove date June 2010 Infobox Disease Name PAGENAME Image Caption DiseasesDB 32948 ICD10 ICD9 ICD9 277.87 ICDO OMIM 603041 MedlinePlus eMedicineSubj eMedicineTopic MeshID Mitochondrial neurogastrointestinal encephalopathy syndrome MNGIE , also called myoneurogastrointestinal encephalopathy syndrome or POLIP syndrome , ref name omim OMIM 603041 ref is a rare autosome autosomal dominance genetics recessive ref name mnear cite pmid 19056268 ref mitochondrial disease usually appearing between the second and fifth decades of life. Unlike typical mitochondrial diseases caused by mitochondrial DNA mtDNA mutations, MNGIE is caused by mutations in the ECGF1 TYMP gene, which encodes the enzyme thymidine phosphorylase. ref name omim A secondary form of MNGIE, called MNGIE without leukoencephalopathy , can be caused by mutations in the POLG gene. ref name omim Signs and symptoms MNGIE is a multisystem disorder. Gastrointestinal symptoms may include gastrointestinal dysmotility possibly resulting in pseudo obstruction in which the muscular contractions peristalsis of the gastrointestinal tract become inefficient causing malabsorption . Borborygmi stomach rumbling , early satiety, diarrhea, constipation, gastroparesis , nausea, vomiting, weight loss, diverticulitis . Neurological symptoms may include diffuse leukoencephalopathy , peripheral neuropathy , and myopathy .Ocular symptoms may include retinal degeneration, ophthalmoplegia paralysis of eye muscles , ptosis drooping eyelids . Those with MNGIE are often thin, experiencing continuous weight loss. References reflist External links http www.ncbi.nlm.nih.gov bookshelf br.fcgi?book gene&part mngie GeneReview NIH UW entry on Mitochondrial Neurogastrointestinal Encephalopathy Disease Mitochondrial diseases Inborn errors of purine pyrimidine metabolism DEFAULTSORT Mitochondrial Neurogastrointestinal Encephalopathy Syndrome Category Mitochondrial diseases Category Rare diseases Category Syndromes Category Autosomal recessive di ... more details
protein Name mitochondrially encoded tRNA valine caption image width HGNCid 7500 Symbol MT TV AltSymbols MTTV EntrezGene 4577 OMIM RefSeq NC 001807 UniProt PDB ECnumber Chromosome MT Arm Band LocusSupplementaryData Mitochondrially encoded tRNA valine also known as MT TV is a transfer RNA which in humans is encoded by the mitochondrion mitochondrial MT TV gene . ref name pmid7219534 cite journal author Anderson S, Bankier AT, Barrell BG, de Bruijn MH, Coulson AR, Drouin J, Eperon IC, Nierlich DP, Roe BA, Sanger F, Schreier PH, Smith AJ, Staden R, Young IG title Sequence and organization of the human mitochondrial genome journal Nature volume 290 issue 5806 pages 457 65 year 1981 month April pmid 7219534 doi 10.1038 290457a0 url ref MT TV is a small 69 nucleotide RNA human mitochondrial map position 1602 1670 that transfers the amino acid valine to a growing polypeptide chain at the ribosomal ribosome site of protein synthesis during translation genetics translation . References Reflist External links http www.ncbi.nlm.nih.gov books NBK1173 GeneReviews NCBI NIH UW entry on Mitochondrial DNA Associated Leigh Syndrome and NARP Mitochondrial enzymes genetics stub ... more details
inline date April 2010 Human mitochondrial genetics is the study of the genetics of the DNA contained ... to use, and are hence referred to as the powerhouses of the cell. Mitochondrial DNA mtDNA is not transmitted through nuclear DNA nDNA . In humans, as in most multicellular organisms, mitochondrial DNA is inherited only from the mother s ovum . ref see Paternal mtDNA transmission ref Mitochondrial ... of mitochondrial DNA genetic code codes for functional mitochondrial proteins, and therefore most mitochondrial ... disorders. Mitochondrial function and genome Because they provide 36 molecules of ATP per glucose ... to all higher organisms for sustaining life. The mitochondrial disease s are genetic disorder s carried in mitochondrial DNA, or nuclear DNA coding for mitochondrial components. Slight problems with any ... is very difficult 10 protons to enter the mitochondrial matrix along their concentration gradients ... lead to a number of diseases. Genome see Mitochondrial DNA Mitochondrial DNA mtDNA is present in mitochondria ... about 13 of them are coded on the mitochondrial DNA. Most of the 3000 proteins are involved in a variety ... copies of the mitochondrial genome are carried by each mitochondrion 2 10 in humans , mitochondrial ... of the 9 genes on the light strand code for mitochondrial tRNA molecules. Human mtDNA consists of 16,569 ... the origins of replication of both heavy and light strands. The entire human mitochondrial DNA ... of nucleotides of a human mitochondrial DNA molecule 16,569 , as much as 0.42 of the mtDNA varies ... exceptions mitochondrial genetics includes some of these. For most organisms the stop codon s are UAA ... mitochondrial mRNA. There are many other variations among the codes used by other mitochondrial ... will be the same in their mitochondrial genome . Using these techniques, it is estimated that the first ... thumb right Mitochondrial inheritance patterns alt unaffected mother and affected father ... Because mitochondrial disease s diseases due to malfunction of mitochondria can be inherited both ... more details
Image Mitochondrie.svg thumb 300px right Mitochondria structure 1 inner membrane, 2 outer membrane, 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 mitochondrial membrane 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
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
Refimprove date January 2008 Infobox Disease Name Mitochondrial trifunctional protein deficiency Image Caption DiseasesDB 34111 ICD10 ICD9 ICDO OMIM 609015 MedlinePlus eMedicineSubj ped eMedicineTopic 1284 MeshID Mitochondrial trifunctional protein deficiency is an autosomal recessive fatty acid oxidation disorder ref name faod cite journal author Solish JO, Singh RH title Management of fatty acid oxidation disorders a survey of current treatment strategies journal J Am Diet Assoc. volume 102 issue 12 pages 1800 1803 year 2002 pmid 12487544 doi 10.1016 S0002 8223 02 90386 X ref that prevents the body from converting certain fats to energy, particularly during periods without food . People with this disorder have inadequate levels of an enzyme that breaks down a certain group of fats called long chain fatty acids. Signs and symptoms Signs and symptoms of mitochondrial trifunctional protein ..., breathing difficulties, coma, and sudden death. Signs and symptoms of mitochondrial trifunctional ... Image autorecessive.svg thumb right Mitochondrial trifunctional protein deficiency has an Recessive ... mitochondrial trifunctional protein deficiency. These genes each provide instructions for making part of an enzyme complex called mitochondrial trifunctional protein. This enzyme complex functions in mitochondria, the energy producing centers within cells. As the name suggests, mitochondrial trifunctional ... in the HADHA or HADHB genes that cause mitochondrial trifunctional protein deficiency disrupt all three ..., heart, and muscles. This abnormal buildup causes the other signs and symptoms of mitochondrial trifunctional protein deficiency. Pathophysiology The mitochondrial trifunctional protein, composed of 4 alpha and 4 beta subunits, catalyzes 3 steps in mitochondrial beta oxidation of fatty acids long ... in the nervous system that affect arms and legs peripheral neuropathy . Episodes of mitochondrial ... and mortality. Epidemiology Mitochondrial trifunctional protein deficiency is a rare disorder its ... more details
humans . She is commonly called Mitochondrial Eve . genetic genealogy Evolutionary relationship ... van Oven M, Kayser M title Updated comprehensive phylogenetic tree of global human mitochondrial ... 18853457 doi 10.1002 humu.20921 ref and subsequent published research. Macro haplogroup L mtDNA L Mitochondrial ... of Eve Bryan Sykes had claimed there were seven major mitochondrial lineages for modern Europeans ... Human mitochondrial molecular clock Cambridge Reference Sequence haplogroup Human mitochondrial ... references External links Mitochondrial Phylogenetic Trees Ian Logan s http www.ianlogan.co.uk mtDNA.htm Mitochondrial DNA Site Mannis van Oven s http www.phylotree.org PhyloTree.org cite journal author ... 08 02.jpg Mitochondrial haplogroup skeleton Vincent Macaulay s http www.stats.gla.ac.uk vincent founder2000 motif.html Mitochondrial haplogroup motifs Cambridge DNA s https www.cambridgedna.com ... pdf 43026 doron.pdf Commons category Human mtDNA haplogroups DEFAULTSORT Human Mitochondrial ... more details
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